Lymphangioleiomyomatosis (LAM) represents a rare neoplasm affecting almost exclusively women of reproductive age. This condition mainly affects the lungs, but extrapulmonary locations such as the pelvis and the retroperitoneum are possible. Clinical evaluation and ultrasound imaging are usually non-specific, and the diagnosis is obtained through surgical excision and histopathological examination. We report a very rare case of abdominal LAM in a young female patient. A thorough literature review of this rare condition with emphasis on gynecologic implications will be presented. The patient was referred for gynecologic consultation due to pelvic pain and infertility. Unfortunately, despite prompt diagnosis and treatment, the course of the disease was severe and led to patient's exitus in a short time. We encountered an extremely rare deadly pathology mimicking a very common gynecologic condition. The gynecologist must always be alert of possible unexpected conditions that will require prompt attention.
Keywords: Lymphangioleiomyomatosis; bilateral hydrosalpinx; chronic pelvic pain; infertility; laparoscopy.