Sebaceous lymphadenoma (SLA) is a rare benign tumor of the salivary gland that commonly arises in the parotid gland in adults. It is rarely diagnosed correctly preoperatively. In addition, to the best of our knowledge, SLA has not been described yet in the literature in association with Cowden's syndrome (CS). We present an extremely rare case of parotid SLA that was diagnosed preoperatively by fine needle aspiration in a patient with CS.