The natural history of multicystic dysplastic kidney (MCDK) is not well established. We analyzed kidney growth and renal function in 33 children with prenatally diagnosed unilateral MCDK in a long-term study. The mean observation period was 4.9 years with a range of 1-11.6 years. Abnormalities of the contralateral kidney were found in 10 of 33 patients (30%): ureteropelvic junction obstruction (5), ureterovesical junction obstruction (2), and vesicoureteral reflux (3). In 6 children the dysplastic kidney had been removed. Complete involution was observed in 48% and a decrease of size in 33% of 27 dysplastic kidneys. At the time of last examination, 27 of 29 children showed a volume of the contralateral kidney above the normal range (>145%). Hypertrophy of the contralateral kidney, defined as kidney length above 2 standard deviation scores (SDS), was seen in 24% of 33 children at birth, thus showing that hypertrophy of the contralateral kidney starts in utero and continues throughout childhood. The extent of contralateral hypertrophy was independent of associated abnormalities in this study. Mean creatinine was increased in the whole group (mean +1.13 SDS). Calculated creatinine clearance in 21 patients over 2 years was within normal limits, with a median of 102 ml/min per 1.73 m2 (range 84-143). Based on the results of this and previous studies, nephrectomy cannot be recommended in typical cases, but a regular follow-up of these patients seems necessary.