Giant hydronephrosis is rare in children. A 7-year- old male child with giant hydronephrosis due to congenital ureteropelvic junction (UPJ) obstruction is reported. Radiological features and management are briefly discussed. The literature is reviewed KEWORDS: Giant hydronephrosis; UPJ obstruction; Intrinsic. INTRODUCTION Giant hydronephrosis is defined as a kidney containing more than 1L of fluids in its collecting system [1]. The radiological diagnostic criteria are a hydronephrotic renal pelvis that meets or crosses the midline, occupies a hemi-abdomen and extends for a length of five vertebrae or more [2]. The commonest cause is UPJ obstruction which occurs in 80% of causes [2]. Giant hydronephrosis is a slowly progressive disease and a huge abdominal mass or distended abdomen may be the only sign. The differential diagnosis includes ascites, intraperitoneal, and retroperitoneal cysts, pancreatic pseudocyst, and ovarian cyst or tumors [3]. The majority of giant hydronephrotic kidneys are non-functioning and symptomatic, making nephrectomy the procedure of choice [4]. With higher standards of medical care, giant hydronephrosis is now a rare urological entity. The author feels that this case reported here is worth documenting, since the degree of pelvic dilatation was most unusual especially in a child of 7 years.