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We report a case of lymphangiomyomatosis (LAM) which occurred in a twenty six year old woman, presenting with protein losing enteropathy and pancreatic fibrosis due to lymphostasis. Despite having no pulmonary lesion, the patient died quickly. The proliferation of smooth muscle involved the mesenteric, peripancreatic lymphatic channels but also the submucosal lymphatic vessels of the duodenum. Only one other case of LAM presenting with protein losing enteropathy has been reported, in which there were no abnormalities in the intestinal tractus or in the pancreas.