Common variable immunodeficiency (CVID) is a disorder of typically adult-onset hypogammaglobulinemia in the absence of other known causes. Noninfectious gastrointestinal tract manifestations are common, and a subset of patients with CVID present with a severe enteropathy manifesting as severe malabsorption, weight loss, and diarrhea. A 63-year-old man presented with CVID and severe enteropathy who failed to improve with cytomegalovirus therapy, multiple empiric therapies, and corticosteroids. Vedolizumab infusions were initiated with a dramatic, rapid, and durable complete clinical response.
