Nephrotic Syndrome Associated with Hydronephrosis and Unilateral Renal Agenesis

We reported a case of nephrotic syndrome in a child presenting undiagnosed hydronephrosis and unilateral renal agenesis in a 3 years and 9 months old male patient presenting features of nephrotic syndrome, with left hydronephrosis and unilateral renal agenesis. The association of nephrotic syndrome with congenital anomalies is extremely rare. The importance of this unusual combination was discussed respecting the literature.