We presented seven multicystic dysplastic kidney cases treated in our Hospital during the last 15 years. Four children were diagnosed prenatally and three during the first term of life. Five were girls and two boys. We found three contralateral upper urinary tract anomalies. We did five nephrectomies. In two cases because compressive abdominal mass during the neonatal period, in two cases during the first year of life because symptomatic urinary tract infections and one case at the age of 13 months with persistent multicystic dysplastic kidney followed by ultrasound. Two cases were not operated. In one patient with partial regression of the multicystic kidney the family refused operation. We have lost follow-up in the last patient.