Enamel cell tumor of jaw bone is a central epithelial tumor of jaw bone, which is more common among odontogenic tumors; it occurs mostly in young adults, and is common in the body of jaw bone and jaw angle. This tumor is expansive and has the potential to become cancerous, which can cause functional damage to the jawbone and affect the appearance after removal. This patient also found me after repeated recurrence of enameloblastoma, and I hope that this case sharing will help you to have more understanding of enameloblastoma. Brief description of the case: This male patient is 62 years old and his family accompanied him to come all the way from his hometown. When they received the patient in the outpatient clinic, they could see that his left maxillofacial swelling was already very large and they had already been transferred to many hospitals and were told not to operate in view of the patient’s huge tumor and unsatisfactory general condition. The patient’s condition was unique. The patient was diagnosed with “left mandibular enucleated cell tumor” at the local hospital 20 years ago and underwent “mandibular mass excision + rib flap repair” (details unknown). The tumor recurred 10 years ago, so he went back to the local hospital for ablation surgery. Unfortunately, six months later, the tumor was found to have grown again in the area previously ablated. Moreover, the patient had a history of advanced hepatitis C cirrhosis and liver cancer in the past. After admission, we perfected the relevant examination for the patient. The patient had asymmetry in the jaw and face. The left preauricular area and the left mandibular parapharyngeal area in the mouth as well as the posterior maxillary bulge were obvious, and the surface of the mass was smooth, hard, immobile, and palpable with a ping-pong-like sensation. Under MRI as shown in the figure, the mass was seen to be huge: MR plain + enhanced images were seen: left buccal bulge, left mandibular body and ascending branch were seen to be distensible. Osteolytic bone destruction, bone cortical discontinuity, rough and irregular margins, and surrounding soft tissue mass formation, the upper edge to the left temporal, the lower edge to the lower part of the mandibular chin, the inner edge to the left inferior temporal fossa/parapharyngeal space and the floor of the mouth, the extent of about 63 mm × 66 mm, enhancement mass is not uniformly enhanced, and within it are seen multiple cystic low signal non-enhanced areas of varying size, the left oropharyngeal cavity is narrowed, the left tongue body is compressed and encapsulated The left temporal arch causes partial bone resorption/thinning, and the adjacent left temporal bone has a poorly defined local contour and mild irregular thinning. There was no clear thickening of the local dura after enhancement; enlarged lymph nodes were seen in the left cervical region II with a short diameter of about 13 mm, and multiple small lymph nodes were seen in the remaining bilateral cervical and submandibular regions, the larger one with a short diameter of about 5 mm. The patient’s coagulation function as well as liver function were also poor in the blood examination, with platelets 77*10^9/L; coagulation function: prothrombin international ratio 1.66, activated partial thromboplastin time 53.0s, prothrombin time 19.2s, liver and kidney function: glutamic aminotransferase 70U/L, glutamic oxalacetic aminotransferase 99U/L, total bilirubin 45.4μmol/L. Thus, the patient’s recurrent tumor was huge and involved more anatomical areas for resection, including the left skull base and maxilla, left cheek, near 3/4 of the mandible, left parapharynx. The patient’s recurrence was after multiple surgeries, and the anatomical structure was not clear, thus the surgery itself was difficult, while cirrhosis and postoperative effects of hepatocellular carcinoma made the systemic platelets very low, which increased the risk and difficulty of the surgery. Therefore, we asked the gastroenterology, hepatobiliary surgery, cerebral surgery, ICU and nutrition department to actively consult the patient before surgery and actively regulate the patient’s whole body condition to prepare for the surgery. We performed tracheotomy + enlarged left maxillary/ mandibular/ buccal/ skull base/ oropharynx/ floor of the mouth mass + right lateral femoral flap repair on the selected day. The size of the right lateral femoral flap was 30×12 cm, and we used a huge tissue flap to repair the large defect after tumor resection very well. The operation went smoothly, but the patient was transferred to the ICU after the operation because of the heavy underlying disease. Intraoperative cryopreservation and postoperative routine pathology confirmed that the enamel cell tumor was not malignant. Case notes: Enamel cell tumor is one of the most common odontogenic tumors, which is a central epithelial tumor of the jaw bone, mostly occurring in the mandible of young adults, and although it is benign, it has a certain degree of recurrence and belongs to the category of “critical tumor”. It is very important to ensure the integrity of the envelope, especially if the tumor invades the muscle, it is important to cut the muscle tissue at the level of attachment outside the invaded muscle, otherwise the tumor may spread beyond the surgical anatomical area by contraction of the muscle, which may make it more difficult to operate again or even endanger the patient’s life. In this case, the recurrent tumor affected the skull base area and the left temporalis muscle, therefore, it is likely that the enamel cell tumor spread to the muscle, therefore, we need to emphasize the anatomical scope of the surgery and operate according to the anatomy. At the same time, the patient’s systemic condition was special, with advanced cirrhosis and postoperative hepatocellular carcinoma, and his preoperative platelets were only 77*10^9/L, which was very challenging for the resection of such a huge tumor. The various intraoperative and postoperative stimuli are likely to cause liver failure or even death of the patient. Thus, it is important to inform the patient and his family preoperatively that his enamel cell tumor is already huge and once it enters the skull, chances are that there is no surgical treatment value. Weighing the patient’s tumor and systemic condition, it is necessary for the patient, his family and the doctor to share the responsibility. The patient was refused treatment by several hospitals, and if I also refused to operate on him, it is likely that the patient would have lost the chance of treatment completely. This is what I often say as a surgeon who encounters special cases must have the responsibility, and cannot be limited to routine surgery. Of course, we were well prepared before the operation, but even so, the patient developed hepatic encephalopathy at one point after the operation, thus showing the special nature of this case. Finally, I would like to emphasize that the complete resection of this recurrent and huge tumor cannot be achieved without the surgeon’s own surgical skills and a high degree of responsibility. The design of the tumor resection path, intraoperative bleeding control, and sequential exposure of the anatomical region are all critical to the overall outcome of the surgery and the prognosis of the patient. Thus, I think it is a practice for us as oncologic surgeons to be able to handle such cases well! Grateful for this duty! Grateful for the trust of patients and families!