Celiac disease is characterized by intestinal inflammation and mucosal atrophy that improves on a gluten-free diet. Delayed diagnosis can result in diet-refractory disease known as refractory sprue, which is linked to other disorders (intestinal lymphoma, ulcerative jejunoileitis, mesenteric lymph-node cavitation, collagenous sprue, malignancy) and carries a poor prognosis. Here we report the case of a young woman diagnosed with Marsh stage 3C celiac disease with intestinal occlusion due to intra-abdominal cystic lymphangioma mimicking mesenteric lymph-node cavitation, and hyposplenism. Despite a number of prognostically negative features, the disease has been managed successfully for the past 2 years with a gluten-free diet. The pathogenesis, prognosis and therapeutic implications are discussed.