Agenesis of teeth and ankyloglossia are common human developmental anomalies. Terms like Oligodontia, Anodontia and Hypodontia are used to describe agenesis of teeth. Oligodontia is a rare condition generally defined as agenesis of six or more teeth excluding the third molars. The condition is not frequently documented in Indian children. There is no much documentation on oligodontia with ankylogossia. Ankyloglossia can adversely affect the development of the surrounding structures including the alveolar process, teeth and can impair functions such as mastication, speech, and swallowing. The present article reports a rare case of non-syndromic oligodontia associated with ankyloglossia in an 8-year old male patient. Oligodontia is a relatively rare condition affecting 0.1-0.2% of the population. Our present case is even rare because of its association with ankyloglossia