Abstract Aim: Distribution of hypodontia and hyperdontia in the reported concomitant hypo-hyperdontia (CHH) patients. Materials and Methods: An extensive search of the reported literature from January 1966 to December 2015 was conducted using the “EmBase,” “Google Scholar,” “Medline,” and “PubMed” databases. The search words used were “agenesis,” “concomitant,” “hypodontia,” “hyperdontia,” “hypo-hyperdontia” “concomitant hypo-hyperdontia” and “supernumerary teeth” in different combinations. The citation lists from the included references were subsequently examined, and a hand search was performed in an attempt to find additional data. Detailed analyses of the incidence of hyperdontia and tooth type of hypodontia were evaluated to determine the occurrence of hypodontia and hyperdontia in this condition. Descriptive statistics were carried out using a Chi-square test to determine the occurrence of hypodontia and hyperdontia in CHH, the value of P < 0.05 was considered statistically significant. Results: Overall, 103 individuals were reported on CHH in published English literature. The average age of them was 11.29 years and frequently observed in males (P < 0.05). Overall 63% of teeth were missing in all the cases that were reported on CHH, whereas 37% of supernumerary teeth were documented (P < 0.05). Most of the cases involved are an anterior region of the both arches (57%). Second premolars are the most commonly missing teeth in both maxillary and mandibular arches, and maxillary mesiodens (50%) are frequent supernumeraries in CHH patients. Conclusion: Hypodontia is more common in CHH patients than hyperdontia. Second premolars are the commonly missing teeth while maxillary mesiodens are the frequently reported in CHH patients. Posterior CHH has not been reported.