Background: Developmental disturbances of tongue and teeth can adversely affect the development of the surrounding structures including the palate and alveolar process. These developmental disturbances impair functions such as mastication, speech, and swallowing. Partial anodontia (synonym: oligodontia) is a condition associated with missing six or more permanent teeth. Ankyloglossia or tongue tie is in which the tip of the tongue cannot be protruded beyond the lower incisor teeth. Case Report: We are presenting a rare case of male patient with partial anodonia and ankyloglossia. The patient was advised for prosthesis with respect to missing teeth and frenuloplasty to relieve tongue tie. Conclusion: This case report offer guidelines which can be used by an oral physician for diagnosis and treatment of a tongue restriction resulting from ankyloglossia and partial anodontia.