Short bowel syndrome (SBS) is a rare disorder characterized by severe intestinal dysfunction leading to malabsorption of macronutrients and micronutrients that often results in permanent need of parenteral nutrition support. Patients can develop SBS because of massive intestinal resection or loss of intestinal function and consequently experience significant morbidity and increased healthcare utilization. The remaining anatomy and length of bowel after intestinal resection have important prognostic and therapeutic implications. Because patients with SBS constitute a heterogenous group, management is complex and multifaceted, involving nutrition support, fluid and electrolyte management, and pharmacologic therapies in particular to control diarrhea. Surgical interventions including intestinal transplantation may be considered in selected individuals. Successful care of these patients is best accomplished by a multidisciplinary team that is experienced in the management of this syndrome.