Lymphangioleiomyomatosis is a rare disorder of unknown etiology and occurs almost exclusively in women, usually during reproductive period. Abdominal and lymphatic abnormalities might be accompanied by cystic lung lesions. The case with LAM and tuberosclerosis was reported, but LAM with diabetes insipitus (DI) and genital hypoplasia was not reported before in the literature. In this case we present a 19-year-old female with LAM together with DI and genital hypoplasia.