A case of basal cell adenoma of the submandibular gland in 4-Month-old infant was reported with a review of the literature. The patient complained of right submandibular tumor. The tumor was surgically removed. Histological examination revealed a basal cell adenoma of the submandibular gland. This is a specific type of monomorphic adenoma of the salivary gland that closely resembles basal cell lesions of the skin. Seventy-six cases were reported in the world by 1986. The majority have been occurred in patients over 60 years and only 3 cases were infant. So, our report is an exceptional case.
