Abstract: Sclerosing odontogenic carcinoma (SOC) is an extremely rare disease which is a low-grade malignancy. There are no typical symptoms in the early stage and it is easily ignored. Patients often seek medical attention because the tumor compresses the respiratory tract at the bottom of the mouth or causes facial/voice changes. This disease has many similarities with clear cell odontogenic carcinoma, calcifying epithelial odontogenic tumor with Langerhans cells, mandibular sclerosing osteomyelitis and other diseases in clinical manifestations, imaging and pathological examination, which interferes with early diagnosis. Through literature review, this paper summarizes the progress of diagnosis and treatment of SOC in order to provide reference for clinical diagnosis and treatment.