Agenesis of bilateral maxillary canines is very rare and mesiodens is a commonly occurring supernumerary tooth type. Concomitant occurrence of both hypodontia and hyperdontia is extremely rare and it is a condition of mixed numeric variation in the same individual. The reported prevalence of this condition ranges between 0.002% and 3.1%. The purpose of this case report is to describe a rare occurrence of hypo-hyperdontia involving agenesis of both maxillary canines, mesiodens and associated with taurodontism.