Thirty cases of multicystic dysplastic kidney (MCDK) were diagnosed over 11 years. Nine patients underwent nephrectomy: three for increasing kidney size (classic MCDK) and six because of an inconclusive diagnosis (hydronephrotic MCDK). Of the remaining 21 patients, 19 were followed up for a mean of 33.5 months (range, 2-101 months). Follow-up ultrasound examinations revealed that 16 kidneys did not change in size, one decreased in size after cyst puncture, and two disappeared (one after cyst puncture). This series included one case of non-renin-producing hypertension that was controlled medically, one case of nephroblastomatosis found in the removed dysplastic kidney, and one case of pyelonephritis in the contralateral kidney. When the diagnosis of classic MCDK is made with imaging modalities, the lesion may not have to be removed unless there is growth of the mass during the 1st year of life. Nine percent of these lesions will disappear within the first 3 years of follow-up, and the authors recommend an even longer period of follow-up.