The most common cause of hydronephrosis in the pediatric age group is ureteropelvic junction-type hydronephrosis (UPJHN). Since the advent of widespread maternal ultrasound screening, clinical presentation of hydronephrosis associated with UPJ anomalies has changed dramatically. Today most cases are diagnosed in the prenatal period, and neonates present without signs or symptoms. For those who are not detected at birth, UPJHN eventually presents throughout childhood and even adulthood with various symptoms. Clinical picture of UPJHN highly depends on the presence and severity of obstruction, and whether it affects single or both kidneys. Abdominal or flank pain, abdominal mass, hematuria, kidney stones, urinary tract infections (UTI), and gastrointestinal discomfort are the main symptoms of UPJHN in childhood. Other less common findings in such patients are growth retardation, anemia, and hypertension. UTI is a relatively rare condition in UPJHN cases, but it may occur as pyelonephritis. Vesicoureteric reflux should be kept in mind as a concomitant pathology in pediatric UPJHN that develop febrile UTI. Although many UPJHN cases are known to improve over time, close clinical observation is critical in order to avoid irreversible kidney damage. The most appropriate approach is to follow-up the patients considering the presence of symptoms, the severity of hydronephrosis and the decrease in kidney function and, if necessary, to decide on early surgical intervention.