OBJECTIVE: Detection of congenital urinary system anomalies is an important part of the prenatal ultrasound examination. The present study compares prenatal ultrasonographic findings and postmortem examinations of fetuses and infants with renal and urinary tract anomalies. DESIGN: Criteria for inclusion were an ultrasound examination at the National Center for Fetal Medicine (Trondheim, Norway) and autopsy performed during the period 1985-94. Results from the ultrasound examination and autopsy regarding urinary system anomalies were categorized according to the degree of concordance. RESULTS: Urinary system anomalies were found in 112 (27%) of 408 fetuses with congenital anomalies. The renal and/or urinary tract anomaly was the principal reason for induced abortion or cause of death in 50 cases (45%). In 97 (87%) of the 112 cases there was full agreement between the ultrasound observations and the autopsy findings. In five cases the autopsy revealed minor findings not mentioned in the ultrasound report. The main diagnosis was thus correct in 102 cases (91%). In four cases major autopsy findings had not been found by ultrasound examination; in another four, none of the autopsy findings were suspected by ultrasound, and in two, minor ultrasound findings were not confirmed at autopsy. CONCLUSIONS: The accordance between ultrasound diagnoses and postmortem examinations proved to be satisfactory. The close co-operation between ultrasonographers and perinatal pathologists is mutually beneficial. In addition to complementing prenatal diagnosis, postmortem examination is of vital importance for the quality control of ultrasonography in fetal diagnosis and plays an important role in genetic counseling.