Birth prevalences of congenital anomalies in the American Indians of British Columbia are compared with those of the total British Columbia population. This study is based on data from the British Columbia Health Surveillance Registry for a 16-year period (1966-1981) judged to be the most reliable reporting period in the 35-year history of the registry. The overall congenital anomaly frequency is lower in Indians than in the general population (45 versus 60 per 1,000 livebirths). The Indian rates for individual anomalies are lower than the corresponding general population rates with the exception of orofacial clefting and congenital heart defects. Defects of the central nervous system in both populations are comparable. There is a striking paucity of hypospadias, other anomalies of the genital organs and foot deformities in Indian males. It is suggested that the differences in the congenital anomaly rates between the American Indians and the non-Indians of British Columbia may reflect genetic differences between the two groups, but differences in ascertainment and infant mortality probably also play a role.