Clear cell carcinoma is a rare low-grade carcinoma. It occurs almost exclusively in the intraoral minor salivary glands and represents less than 1% of all salivary gland tumors. It is a new addition in the recent World Health Organization (WHO) classification of salivary gland tumors. The condition follows an indolent course and treatment includes wide surgical excision with or without adjuvant radiotherapy. This case report highlights a very rare case of a 40-year-old female patient with characteristic clinical and radiographic features, diagnosis and management including significant histopathologic features. Combining our case with those reported in literature, a total of 70 cases confirm that clear cell carcinoma is a low-grade malignant neoplasm with distinctive clinical and pathologic features, which highlight the rarity of the condition.