This case report defines a case of isolated oligodontia of 9 and 10 permanent teeth in 9-year-old monozygotic twin sisters and gives information about the possible genetic and environmental etiology, related dental anomalies and treatment options. The twins have a negative family history of hypodontia and oligodontia in their parents, as well as their paternal and maternal grandmothers and first cousins. No other dental anomalies could be detected in either of the twins. With the occurrence of similarly located tooth agenesis, except for one tooth, in monozygotic twins, one may consider the influence of genetic and/or environmental factors in their etiology. Hereditary relationships associated with oligodontia could help the clinicians to predict the possibility of its occurrence in other family members and in the next generations. However, clinicians should consider oligodontia when it is not hereditary.