Leiomyomas account for less than 0.43% of all bladder tumors. To date only about 250 cases have been reported in the literature. Pulmonary lymphangioleiomyomatosis (LAM) is a rare lung disease featuring abnormal proliferation of atypical smooth muscle cells around bronchovascular structures. We describe a woman with LAM who presented with pelvic pain and was found to have a leiomyoma of the bladder wall. Although urologic manifestations of pulmonary LAM are uncommon, they should be considered in the differential diagnosis of a patient who presents with a pelvic mass and a history of LAM.