Multicystic dysplastic kidney (MCDK) is the most common cystic kidney disease in children. Renal abnormalities occur in the contralateral kidney in 20-57% of cases. The prognosis of MCDK depends primarily on the status of the opposite kidney. We describe a case of unilateral MCDK and contralateral hypoplasia presenting with renal failure during the neonatal period. Abdominal ultrasound showed a multiple non-communicating cystic mass measuring 3.5×1.8 cm in the left kidney. The right kidney was small measuring 2.5×1.3 cm with increased echogenicity. (superscript 99m)Tc-mercaptoacetyltriglycine renal scintigraphy showed an absence of uptake and flow of the left kidney and a decreased uptake of the right kidney. At 1.5-year of follow up, the MCDK had a partial involution, and the patient had chronic renal failure with growth retardation. In conclusion, children with unilateral MCDK and contralateral renal hypoplasia may develop renal failure early in their lives. The optimal conservative treatment of MCDK patients with chronic renal failure requires close monitoring of each patient's clinical and laboratory status to slow the disease progression and to induce healthy growth.