Short bowel syndrome occurs subsequent to anatomical and/or functional loss of mainly small bowel. This often-devastating disease leads to weight loss and immune dysfunction. Proper medical management involves adequate substitution and maintenance of fluid, electrolytes, and nutrients. Although several pharmacological therapies such as clonidine, growth hormone, or octreotide have shown promising results in short bowel syndrome, optimal nutritional management is the most important factor in these patients. If enteral nutrition is possible, diet should consist mainly of fat, followed by protein, and less intake of carbohydrates. Supplementary nonprocessed cereals may be beneficial in a certain subgroup of patients. With the recent developments in medical therapy, a balanced diet may allow many patients to become nutritionally autonomous.