We report about a 43-year old woman with relapsing polychondritis, admitted with progressive malaise, fatigue, anorexia and profound weight loss. Two years ago a nasal chondritis with characteristic changes of the nasal profil, scleritis, laryngitis and unspecific general symptoms (fever, fatigue, signs of a systemic inflammation) developed and relapsing polychondritis was diagnosed. The patient was treated initially with azathioprine followed by methotrexate in combination with ciclosporine and glucocorticoids. During the hospitalization her condition slowly worsened, and crampy abdominal pain developed subsequently with massive tenderness, rebound and guarding. The clinically presumed diagnosis of ileus and peritonitis was radiomorphologically confirmed with signs of enteric perforation and a laparatomy was performed. A complete occlusion of the A. mesenterica superior and stenosis of the truncus coeliacus were diagnosed followed by a revascularization with an aorto-mesenteric bypass and subsequent resection of the necrotic ischemic ileum. An association with various autoimmune disorders including vasculitic syndromes is well known for relapsing polychondritis. The case demonstrated a progressive mesenteric ischemia with the acute exacerbation caused by a vasculitic mesenterial occlusion. The unusual presentation should be considered in the differential diagnosis of uncommon abdominal symptoms during the course of relapsing polychondritis.