本文敘述一位45歲的女性病患，因腰酸長達17個月之久，後來被發現右側腎盂旁有囊腫，同時造成了同側腎臟無功能。在做了腎臟切除手術後，病理才發現，是例罕見的囊腫性淋巴管瘤所造成；所以提出來報告，並作文獻上的回顧。

A 45 yr-old woman with a history of flank soreness for 17 mo, was found to have a parapelvic cyst of the right kidney with hydronephrosis. Non-functioning kidney was discovered at our OPD. She received nephrectomy, and the pathologic findings revealed that there was a large transparenchymal paper-thin-walled unilocular cyst, measuring 3.6× 2.5× 2.3 cm in size, located over the parapelvic region, beneath the ureteropelvic junction (U-P junction), with clear fluid content. All microscopic findings were compatible with cystic lymphagioma. Two tiny subcapsular angiomyolipomas were also found coincidentally. We present this case and review the literatures.