Autoimmune enteropathy is a rare chronic diarrheal disease of infancy. Clinicopathologically, this entity is characterized by chronic secretory diarrhea, villous atrophy with crypt hypoplasia of a small intestine and/or associated autoimmune disorders, and absence of severe immunodeficiency. For the confirmation of diagnosis, antienterocyte autoantibody should be delineated. The treatment of choice of this disorder is immunosuppression. We has been experienced a case of autoimmune enteropathy without autoimmune disorders in a 10-month-old male infant. He developed protracted diarrhea from 5 months of his age and has been appeared to be failure to thrive. Antienterocyte autoantibody was demonstrated by immunohistochemistry and western blotting. He was successfully treated with corticosteroid and FK506. This is the first case report of autoimmune enteropathy without autoimmune disorders in Korea.