Presence of red blood cells (RBCs) in urine is hematuria that even in microscopic amounts alarms the patient and parents of the patient, and often prompts physician for many laboratory investigations. Hematuria can be red, dark or cola colored or brown known as macroscopic hematuria, and when it is not visible to unaided eye, it is known as microscopic hematuria. RBCs in urine is one of the most important signs of genitourinary tract disease; however, it is almost never a cause of anemia, since few drops (1 mL) of blood can turn 1 L of urine into red colored urine. Overall the physician should be alert enough not to overlook serious conditions like neoplasms and underlying bleeding disorder, to avoid unnecessary and often expensive laboratory studies. This article provides an approach to the evaluation and management of hematuria in children, and the detection of preventable and treatable conditions at the earliest to limit the disease progression, and overall reduction in cost, energy and anxiety.