Atresia of the submandibular duct orifice is a rare developmental anomaly. Early identification and treatment of atresia of the submandibular duct orifice prevent feeding and breathing difficulties, dilatation of the submandibular duct, submandibular gland infection, or pressure atrophy. Imperforate submandibular duct should be distinguished from duplication abnormalities of the duct and other cystic lesions in the floor of the mouth. To date, magnetic resonance imaging findings of the atresia of the submandibular duct orifice have not been reported. The aim of this study was to describe clinical and radiologic evaluation of an infant with atresia of the bilateral submandibular duct orifices.