Autoimmune enteropathy (AIE) is characterized by protracted diarrhea, malabsorption, immunomediated damage to the intestinal mucosa, and unresponsiveness to changes in diet. The disease is mainly manifested in the small intestine. Lymphocyte deposits are present on the mucous membrane, and anti-enterocyte or anti-goblet cell antibodies have been described in the majority of affected persons. AIE occurs primarily in infants. Immunosuppressive drugs have been used with varying success. The prognosis of AlE is dependent on the degree of severity of the damage to the intestinal mucosa and extraintestinal symptoms and diseases associated therewith.