A 22-year-old woman presented with left pelvic pain and mass. Ultrason
ography confirmed a multilocular left adnexal mass containing cysts of
varying sizes. The patient had no pulmonary symptoms at the time of p
resentation. The mass was surgically excised and pathologic diagnosis
of lymphangioleiomyomatosis (LAM) was made. Subsequently, she develope
d hemoptysis and pleural effusion. High-resolution computed tomography
of the chest showed findings consistent with LAM. Early diagnosis and
treatment for LAM was possible in this atypical case.