Lymphangioleiomyomata were previously referred to as lymphangioleiomyoma, cystic hydatid tumor, and other types. The new classification now refers to lymphangioleiomyomata collectively as lymphangioleiomyomata, and according to their histological structure, they are divided into three types: macrocystic, microcystic, and mixed types of both. The incidence of lymphangioleiomyomatosis is about 1.2 to 2.8 per 1,000, and the incidence is comparable in both sexes. Most of them are present at birth, and a few show clinical symptoms at an older age. The neck is a common site for the development of lymphangioleiomyomatosis. The clinical symptoms depend on the type, extent and depth of the lesion and the degree of surrounding fibrosis. Microcystic lesions are most often found in the oral mucosa of the lips, cheeks, and tongue, and appear as small, soft, yellow, round, cystic nodular or punctate lesions that are isolated or scattered, usually communicating with deep subcutaneous or submucosal lymphatics, occasionally with venous or microvenous malformations, and presenting clinically as small red and yellow blister-like projections. Deep tissue lesions occur in the soft tissue of the lower 2/3 of the face of the lips, cheeks, tongue, and ears. The depth of involvement varies and often results in hypertrophy of the affected area, causing deformities such as megalingualism, macrolabialism, etc., sometimes accompanied by hypertrophic deformities of the jaws. Lymphatic duct deformity may be accompanied by bone hypertrophy deformity and bone morphology abnormalities. For example, mandibular hypertrophy, mandibular protrusion and malocclusion. Localized macrocystic lesions occur in the submandibular, floor of the mouth, parotid area, and upper cervical region, with single or multiple cysts, usually multi-housed cystic cavities, spaced apart from each other and containing clear, yellowish, watery fluid. When accompanied by bleeding or infection, the fluid may be bloody or purulent. The lesions vary in size, have normal surface skin color, are filled, and are soft and volatile on palpation. Unlike deep venous malformations, the postural shift test is negative, but the transillumination test is positive. The diagnosis of lymphatic duct malformation is not difficult based on the history and clinical presentation. To determine its location and size, ultrasound and magnetic resonance can be used to assess the blood supply and extent of the lesion in addition to routine examinations. Occasionally, lymphatic vascular malformations may resolve on their own, possibly due to a short circuit between the venous-lymphatic system. Rarely, clinicians misdiagnose subcutaneous self-exfoliating hemangiomas as lymphatic vascular malformations, and the differential diagnosis between the two is sometimes quite difficult. Surgery was once the treatment of choice for lymphatic duct malformations, but extensive lymphatic duct malformations are often not completely resected and leave significant postoperative scarring. In recent years, with the further understanding of the histopathological characteristics of lymphatic duct malformations and the advancement of technology, the staging and treatment of oral and maxillofacial lymphatic duct malformations have also been innovated. There are many methods for the treatment of lymphatic duct malformation, each with its own advantages and shortcomings. We have received good therapeutic results by using Pingyangmycin treatment and combined with surgical resection for a long time. Pingyangmycin injection treatment Pingyangmycin is an anti-tumor antibiotic screened from 15 types of bleomycin fractions produced by Streptococcus pingyangensis. Its mechanism of action is mainly to form free radicals with iron complexes, which act on DNA to break it down and cause DNA single-strand breakage, and also inhibit the production of tumor blood vessels. Most of the lymphatic duct malformations in children’s maxillofacial area are isolated lymphatic duct masses, which are not directly connected with the systemic lymphatic system or vascular system, and through the injection of Pingyangmycin, it can maintain high concentration and stay in the lesion for a long time, which can inhibit the growth of lymphatic endothelial cells on the one hand and fibrosis of the lesion interstitium on the other hand, and the two synergistic effects can inhibit the growth of the lesion and make it shrink or even disappear. Pingyangmycin injection has good therapeutic effect on small and medium-sized microcystic lymphatic duct malformation in dorsum of tongue, ventral tongue, lip, cheek, floor of mouth, palate, etc. Local injection of Pingyangmycin alone can cure it. For microcystic lymphatic duct malformations deep in the tissue, small doses of multiple injections of Pingyangmycin can also have a remarkable effect in controlling or shrinking the lesions. It is generally advocated to start treatment after the age of 3 years, and the dose and number of injections should not be excessive to avoid tissue development defects. The maximum dose for adults is 8 mg for 1 time, and the dose for children should be reduced as appropriate. Larger areas need to be treated in parts and sites, with repeated injections at intervals of 3 to 4 weeks, with 3 to 5 times as a course of treatment. Chest X-ray and blood picture should be reviewed regularly during treatment to observe any pulmonary fibrosis and blood picture changes. Adverse reactions: Rash, hypothermia, loss of appetite and rare anaphylaxis may occur in a few cases. To avoid serious consequences, clinicians should be fully prepared. Allergic reactions usually occur after repeated local injections of Pingyangmycin, which may accumulate in the body and trigger allergic reactions to the allergen when the drug is used again. Therefore, when using Pingyangmycin treatment, patients should be consulted about their medication history and personal allergy history, and the patient’s mental status should be closely observed and resuscitation measures should be provided during the injection process. The advantages of Pingyangmycin treatment for lymphatic duct malformation: treatment is not restricted by age, easy to use, fast and effective; local non-invasive, no surgical scar, easily accepted by patients’ families. Avoid damaging the vascular nerve and other tissues around the lesion, and also reduce the recurrence rate after treatment. It can be repeatedly injected, and the course of treatment is short. After local injection, the whole body absorbs less drug and has fewer side effects, which is a safe and effective method worth promoting. Pingyangmycin has no immunosuppressive effect and almost does not inhibit the hematopoietic function of bone marrow. Therefore, it can be the treatment of choice for oral and maxillofacial lymphatic duct malformation. For the lesions that are difficult to be removed by surgery and remain and the cases of recurrence after surgery, the efficacy can also be further improved by Pingyangmycin injection.