We report the long-term clinical results of conservative management of children with unilateral multicystic dysplastic kidneys (MCDK). Between 1989 and 2002, 43 children with MCDK detected by prenatal ultrasonography were prospectively followed. At birth, ultrasonography confirmed the prenatal findings in all cases. Patients underwent a radioisotope scan and micturating cystogram in order to confirm the diagnosis and to exclude other uropathies. Follow-up ultrasound (US) examinations were performed at 6-month intervals during the first 2 years of life and yearly thereafter. The mean follow-up time was 42 months (range 12-156 months). Two children developed hypertension during follow-up. In total 257 US scans were performed. The mean number of US scans per patient was 6 (range 3-10). US scans demonstrated partial involution of the MCDK in 30 (70%) cases and complete involution in 8 (19%). The absolute MCDK length remained almost unchanged in 5 children (11%). The estimated median time of complete involution of the MCDK was 122 months [95% confidence interval (CI)=86-158 months]. A total of 33 (76.7%) contralateral kidneys underwent compensatory hypertrophy, reaching a renal length above the 95th percentile during follow-up. The estimated median time for the occurrence of compensatory hypertrophy was 30 months (95% CI=15-45 months). In conclusion, the natural history of MCDK is usually benign but patients must have long-term follow-up with US scans and blood pressure measurements.