Renal disease is a major cause of morbidity and mortality. Pediatric patients with renal disease, especially younger ones may present with nonspecific signs and symptoms unrelated to the urinary tract. Pediatricians, therefore, should be familiar with the modes of presentation of renal disease and should have a high index of suspicion of these conditions. Affected patients may present with signs and symptoms of the disease, abnormal urinalysis, urinary tract infection, electrolyte and acid-base abnormalities, decreased renal function, renal involvement in systemic disease, glomerular and renal tubular diseases, congenital abnormalities, and hypertension. Pediatricians may initiate evaluation of renal disease to the extent that they feel comfortable with. The role of the pediatrician in the management of the child with renal disease and guidelines for patient referral to the pediatric nephrologist are presented.