Lymphangioleiomyomatosis (LAM), a rare multisystem disease affecting mainly young women, is characterized by an abnormal proliferation of smooth muscle-like cells in the lungs. We report a case of endometriosis with chronic pelvic pain due to pelvic LAM. A 41-year-old gravida 1, para 1 woman had been experiencing intermittent left pelvic pain for several years. She also complained of dyspnea on effort 2 years previously, and was diagnosed with pulmonary LAM. Abdominal magnetic resonance imaging showed a right ovarian endometriotic cyst and a left pelvic mass. She was referred to our hospital for the treatment of pelvic pain; she underwent laparoscopic cystectomy of the right ovarian endometriotic cyst. Her left pelvic cyst was found in the retroperitoneal space, and biopsy confirmed the diagnosis of LAM. Therefore, we suggest that LAM localized in the pelvis should be considered when a patient with pulmonary LAM presents with pelvic pain or abdominal distention.