This study reports our experience in the conservative management of multicystic dysplastic kidney (MCDK) disease. Between 1992 and 2003, 35 children, 18 male (51.4%) and 17 female (48.6%), were diagnosed with MCDK and prospectively followed at the Riyadh Armed Forces Hospital. Static scintigraphy with dimercaptosuccinic acid and micturating cystourethrogram were performed in all patients. Ultrasonography (US) examinations were conducted every 3 months, and then annually. Mean age at diagnosis during the postnatal period was 2.4 days. Median follow-up time was 33.75 months. Follow-up US examinations showed partial involution in 17 (48.6%), complete involution in 13 (37.1%), and 5 (14.3%) showed an unchanged size without any pathological manifestations. The mean age at complete or partial involution of the lesion was 43.7 months. No child developed complications such as hypertension or tumors, and all maintained normal growth and development. In conclusion, MCDK lesions are often benign, and the affected kidneys tend to show involution with time.