Abstract
Sclerosing odontogenic carcinoma (SOC) is a primary intraosseous carcinoma of the jaw that was listed as a separate entity for the first time in the latest version of the World Health Organization classification of Head and Neck Tumors (2017). In this report, we present a case of SOC involving a circuitous diagnostic process because of the inadequately detailed biopsy findings and inherent impression based on the imaging manifestations. Through an extensive literature review, the histopathological and immunohistochemical features of the disease were briefly summarized. Radiological findings of SOC have been characterized in detail, and an imaging classification scheme has been proposed to further discuss the diversity of radiographic features. Due to the rarity of the disease, a comprehensive understanding of SOC is needed, and close collaboration between clinicians, radiologists, and pathologists is crucial to avoid misdiagnosis.