ABSTRACT Celiac disease (CD) is an autoimmune enteropathy with recognized extraintestinal manifestations. We describe a 55‐year‐old man with long‐standing, well‐controlled CD who developed progressive shortness of breath and cough. Imaging was indeterminate for usual interstitial pneumonia (UIP), and a surgical lung biopsy demonstrated a UIP pattern. Autoimmune serologies were negative, and no alternate etiologies for UIP were identified. With intermittent adherence to a gluten‐free diet and medical management including nintedanib, his disease progressed, ultimately leading to lung transplantation. This case raises the possibility of an association between CD and UIP of uncertain aetiology and should prompt further discussion as the relationship remains unclear. To contextualize this observation, we conducted a systematic review of published case reports evaluating pulmonary manifestations of celiac disease.