Short bowel syndrome (SBS) is characterized by a malabsorptive state. It is conceivable that the coexistence of SBS and end-stage renal disease can lead to severe metabolic acidosis; however, such a condition has rarely been documented. We herein describe the case of a 64-year-old man with SBS who required maintenance hemodialysis. Persistent metabolic acidosis and mineral and bone disorders should be of particular concern in hemodialyzed patients with SBS.