Lymphangiomyomatosis is a rare disease affecting exclusively women usually in child-bearing age. It is characterized by proliferation of an atypical smooth muscle involving the pulmonary, mediastinal and retroperitoneal lymph system. The case reported here concerns a 53-year old woman presenting with an interstitial pulmonary lesion giving a characteristic image at CT. The patient also had recurrent chylothorax and a renal angiomyolipoma explored by CT and magnetic resonance imaging. The diagnosis of lymphangiomyomatosis was made on a cluster of arguments, without having recourse to surgical lung biopsy.
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