Autoimmune enteropathy is an uncommon cause of chronic diarrhea rarely seen in adults. The disease is secondary to an autoimmune process in the gut that leads to villous blunting and subsequent watery diarrhea, abdominal pain, and severe weight loss. The disease has only been described in 37 adults prior to our case, and variable treatment success has been documented with steroids, immunomodulators, and TNF-α inhibitors. This case is the first to show success in treating autoimmune enteropathy with vedolizumab and provides physicians with an additional therapeutic option when limited by a patient's comorbidities and side effects of other drugs.