Congenital absence of the major salivary glands is an infrequent disorder. Clinically, patients may be asymptomatic or may present with dryness of the mouth, difficulty in chewing and swallowing, and dental caries. The absence of the submandibular gland may be associated with hypertrophy of the contralateral submandibular gland. We report a case of ipsilateral sublingual hypertrophy associated with unilateral submandibular aplasia.