We report on a rare case of a 26-year-old man with neurofibromatosis type I who presented with cramping abdominal pain caused by stones within a horseshoe kidney. He also showed extensive neurofibromas in his neck, thorax, and abdominopel-vic cavity. He had undergone excision of a subcutaneous neurofibroma from his left flank a few years prior. Treatment by extracorporeal shock wave lithotripsy (ESWL) was administered twice. Stones were removed completely and his symptoms were resolved.