We report the case of a 16-year-old male patient with steroid-dependent nephrotic syndrome who developed ischemic colitis. He was diagnosed as having nephrotic syndrome at 10 years of age and had been administered steroid, cyclosporine A, and mizoribine for 7 years. He presented with severe abdominal pain 5 days after intravenous methylprednisolone pulse therapy; thereafter, massive bloody diarrhea developed. Abdominal ultrasonography and computed tomography revealed a marked thickening of the wall of the transverse colon. Colonoscopy confirmed the diagnosis of ischemic colitis. This is the first report of the development of ischemic colitis in a pediatric patient with nephrotic syndrome.