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Clear cell odontogenic carcinoma (CCOC) is a very rare malignant neoplasm of jaw, with a significant clear cell component. It is nearly three times as common in the mandible with distinct female predominance. Past, identified as locally invasive tumor, CCOC is now considered to be malignant neoplasm, showing distant and regional lymph node metastasis. CCOC is histopathologically characterized by large glycogen-rich clear cells, often intermixed with eosinophilic cells. When diagnosing CCOC, ruling out salivary gland tumors, such as mucoepidermoid carcinoma or clear cell adenocarcinoma is important because they have overlapping histologic features with CCOC. Here in, we report a case of CCOC involving the right maxillary sinus of a 72-year-old female.