Chemotherapy is effective in mucinous adenocarcinoma of the appendix. Chemotherapy includes intraoperative and postoperative chemotherapy as well as neoadjuvant chemotherapy. During the operation, fluorouracil injection can be used to flush the abdominal cavity as prescribed by doctor, or drugs such as fluorouracil injection and oxaliplatin injection can be used for postoperative chemotherapy, which can effectively kill cancer cells. Usually this chemotherapy can slow down the growth of the patient’s tumor, prevent recurrence and metastasis, and can prolong the patient’s survival time. Neoadjuvant chemotherapy is chemotherapy before surgery, which can reduce tumor volume and eliminate micro-metastases, etc. Neoadjuvant chemotherapy can, to a certain extent, enable inoperable patients to get the chance of surgery and reduce the probability of intraoperative dissemination and postoperative metastasis. In addition, appendiceal mucosal adenocarcinoma can be treated by surgical resection of the lesion, which can be done by simple appendectomy and right hemicolectomy. Simple appendectomy is feasible if the tumor is well differentiated, has no lymph node metastasis or venous infiltration, is small in size, and does not invade the submucosa. Right hemicolectomy may also be performed if the disease is early and the cancer only involves the submucosa. The 5-year survival rate is higher in patients with right hemicolectomy than in patients with simple appendectomy. The prognosis of primary right hemicolectomy is better than secondary right hemicolectomy. Patients with mucinous adenocarcinoma of the appendix are advised to go to the hospital in time and follow the doctor’s advice to avoid delay in treatment.