Mucinous epidermodysplasia-like carcinoma accounts for 5% to 10% of salivary gland tumors. According to the degree of differentiation and biological behavior of cancer cells, they are classified into low-grade malignant and highly malignant mucous epidermodysplasia-like carcinoma. The main components of the tumor are mucinous-like cells and epidermis-like cells. Clinical manifestations of mucinous epidermis-like carcinoma occur most frequently in the parotid gland, accounting for more than 70%. The small salivary glands are commonly found in the jaw, while other parts such as posterior molar area, cheek, upper and lower lip are less common. Highly differentiated tumors are often painless masses with slow growth. The tumor size varies, the boundary may be clear or unclear, the texture is moderately hard, and the surface may be nodular. The clinical manifestations of mucinous epidermoid carcinoma are closely related to the degree of clinical differentiation. Highly differentiated type, which accounts for the majority, is usually a painless mass with slow growth and long disease duration. The size of the tumor varies, the boundary is clear, hard, movable, and the surface is smooth or nodular. It may be cystic or solid. If the tumor occurs in the palate or posterior molar area, it can be seen as a pale blue or dark purple mass under the mucosa, with smooth mucosa and soft texture, and a small amount of bloody purple-black fluid can be extracted by puncture. Hypofractionated tumors grow faster and are often associated with pain. The boundary is not clear, diffuse, adherent to the surrounding tissues, and can break down and become infected, forming a long-lasting ulcerated surface with yellowish mucous secretions. Sometimes a salivary fistula can be formed. If it occurs in the parotid gland, facial nerve paralysis and facial muscle twitching may occur when the facial nerve is involved. If it occurs in the palate, it may destroy the hard palate. Treatment measures for mucinous epidermoid carcinoma are mainly regional radical resection of the primary site. To prevent recurrence, surgery should be performed to remove the tumor within the normal tissue 1 cm away from the tumor. For the first surgical treatment of highly differentiated mucous epidermoid carcinoma of the parotid gland, regardless of the disease stage, total parotidectomy with preservation of the facial nerve is generally used; for the low differentiated type with more chances of infiltrating the facial nerve, if the facial nerve is involved, total parotidectomy with sacrifice of the facial nerve should be performed; if the length of the facial nerve is larger, a nerve graft can be done after removing a section of the nerve. For mucous epidermis-like carcinoma occurring in the submandibular gland, submandibular triangle dissection should be performed. For those occurring in the palate, partial maxillectomy should be performed. If the tumor has invaded the surrounding tissues, enlarged resection should be done. The rate of regional lymph node metastasis in mucinous epidermodysplasia-like carcinoma is low. Except for the low-differentiated type, cervical lymph node dissection can be considered, and selective cervical lymph node dissection is generally not performed in highly differentiated cases. Mucinous epidermoid carcinoma is not sensitive to radiation therapy, but it can be used in conjunction with radiation therapy after surgery for low differentiation type, which may improve the efficacy or reduce recurrence.