Three cases of odontodysplasia are described, and the literature is reviewed with reference to proposed etiologic mechanisms underlying the pathogenesis of the disorder. The distribution of affected teeth in all four quadrants of one patient is offered as evidence against a somatic mutation as an etiologic mechanism. As infants all three patients had vascular nevi in the facial skin overlying the area where defective teeth developed. Three cases from the literature involved similar vascular lesions. Hypoplastic teeth radiographically similar to odontodysplastic teeth occur in the linear sebaceous nevus syndrome. These findings strongly suggest that local vascular defects are involved in the pathogenesis of odontodysplasia.