Study objective. The aim was to study the spectrum of clinical problems and outcomes in infants born at an urban academic hospital. In consequence, as part of the overall study, the incidence of congenital anomalies and the outcomes of affected infants were recorded.Design. This was a prospective, hospital-based study, undertaken on liveborn infants born over a 3-year period, 1 May 1986 to 30 April 1989.Setting. Kalafong Hospital, Pretoria.Main results. A total of 17 351 liveborn infants was examined and the total congenital anomalies incidence was 11 ,87 per 1 000 Iivebirths. The central nervous system was the system most frequently involved (2,30 per 1 000 livebirths), followed by the musculoskeletal system (2,13 per 1 000 livebirths). The commonest individual congenital anomaly was Down syndrome (1,33 per 1 000 Iivebirths), followed by neural tube defects (0,99 per 1 000 livebirths) and ventricular septal defects (0,69 per 1 000 livebirths). In 11 % (2,25 per 1 000 livebirths) of neonatal deaths, infant loss was attributable to congenital anomalies.Conclusions. The incidence of congenital anomalies in black South African neonates, born in an urban setting, is as high as in other First- and Third-World countries, and the incidence of some individual congenital anomalies is higher. This study indicates the need for further research and the establishment of prenatal, genetics and paediatric facilities to manage these problems