Lymphangioleiomyomatosis (LAM) is a rare disorder characterized by proliferation of smooth muscle cells in the lung parenchyma and lymphatic system . The proliferating cells, namely LAM cells, are characteristically stained with monoclonal antibodies for HMB-45 . Lymphangiomyoma, a cystic mass produced as a result of lymphatic obstruction by proliferating smooth muscle cells in the lymphatic system  is observed in 16–21% of patients with LAM [2–4] and is the third most common form of extrapulmonary involvement of LAM after renal angiomyolipoma and enlarged abdominal lymph nodes . We describe a woman who was admitted to our hospital for increasing abdominal distention. LAM was diagnosed and a pelvic lymphagiomyoma was revealed. The radiological features of lymphangiomyoma are discussed, with emphasis on magnetic resonance (MR) images.