Lymphangioleiomyomatosis (LAM) is a rare disease affecting women, classically involving the lungs. However, extrapulmonary manifestations also occur, including renal angiomyolipomas, retroperitoneal lymphangioleiomyomas and extrathoracic lymphadenopathy. The lung disease is hormone-responsive, but no information exists regarding sex hormone responsiveness of abdominal LAM. Here, we report two women with LAM whose abdominal lymphangioleiomyomas increased in size with hormonal changes of the menstrual cycle. This is the first description of abdominal lymphangioleiomyomas exhibiting hormone responsiveness in LAM. We describe these cases and summarize the literature on abdominal LAM. Menstrual cycle variation should be taken into account when assessing response to therapy, both clinically and in research studies.