Odontomas are a common type of odontogenic tumor, usually asymptomatic and mostly detected on routine radiographic examination. An 11-years-old male child with the chief complaint of mobility of deciduous dentition in the upper front region was diagnosed with an odontome with an impacted central incisor, missing lateral incisor and retained deciduous incisors following radiographic analysis. Histopathology revealed a compound odontoma following a conservative enucleation. Odontomas associated with primary dentition, impacted teeth and erupting into oral cavity have been described, but the association with a missing lateral incisor makes this an interesting case report. How to cite this article: Nammalwar RB, Moses J. A Rare Association of Compound Odontome with Missing Lateral Incisor. Int J Clin Pediatr Dent 2014;7(1):50-53.