A 27-year-old female with pulmonary lymphangioleiomyomatosis was referred to us because of the right large renal angiomyolipoma (AML), 10 cm in diameter, which was found by abdominal CT during close investigation of her pulmonary disease. About 6 months earlier she had received operation for pneumothorax and the histological examination disclosed pulmonary lymphangioleiomyomatosis (LAM). Clinically she does not have tuberous sclerosis. Partial nephrectomy was performed. The renal tumor arose from the lower part of the kidney with somewhat exophytic growth. She is now followed at outpatient clinic without recurrence of renal AML. Pulmonary LAM often associates with renal AML. Although pulmonary LAM is a progressive disease and has a poor prognosis, because of the risk of the rupture of renal AML, large renal AMLs should be treated. Recently several cases have been reported for whom nephron-sparing surgery was performed. This case is 10th report of LAM, which is associated with AML in Japan.