肾周囊性淋巴管瘤，又称肾周淋巴管囊肿，是一种罕见的肾良性病变，目前认为淋巴管瘤是由于生长发育期淋巴引流障碍而引发的疾病［1］。淋巴管瘤通常多发生于颈部及腋下，也可出现于腹膜后、纵隔膜、肠系膜、网膜、结肠和盆腔，腹膜后淋巴管瘤只占1%左右，而肾周淋巴管瘤更为罕见［2-3］。

Lymphangioma is a rare, benign mesenchymal neoplasm, which is characterized by nume-rous intercommunicating cystic spaces containing lymphatic fluid. It is considered a congenital disease resulting from the obstruction of regional lymph drainage during the developmental period. Lymphangioma frequently occurs in the cervical neck and axilla, also in the retroperitoneum, mediastinum, mesentery, omentum, colon, and pelvis, rarely in the perirenal space. These tumors usually present in childhood, but infrequently, these also present in adults. Patients often complain of hematuria, flank pain, or abdominal pain. Complications of lymphangioma have been reported to include infection, ruputure, or he-morrhage. There are three types of lymphangioma commonly identified: capillary, cavernous, and cystic. Cystic type is the one commonly found intra-abdominally or retroperitoneally, and may be uniloculated or multiloculated. All these perirenal tumors have a very low incidence, make it difficult to diagnose. Differential diagnosis must be performed with the primary renal lymphoma, urinoma, polycystic kidney, te-ratoma, both benign and malignant tumors, etc. Endoscopic ultrasound guided fine needle aspiration is recommended in some literatures, which may help make diagnosis and further guide subsequent therapeutic strategy. Regarding treatment, surgical excision can be performed via either laparotomy or laparoscopy. And injection of sclerosants into lympahgioma has been described in the literature in nonsurgical candidates. The optimal definitive treatment is total surgical excision. Despite being rare, the tumor has an excellent prognosis. Here, we report a case of a 48-year-old woman with a left renal mass found in an abdominal ultrasonography during a health checkup. In the case presented, abdominal ultrasonography and magnetic resonance urography (MRU) revealed an approximately 11.3 cm×10.6 cm×12.8 cm multilocular cystic mass in the left perirenal space. There was no history of bowel or bladder complaint, either previous illness episodes. Full blood count and kidney function tests were within normal limits. Laparoscopic surgical removal of the cyst was accomplished without incident. A benign cystic perirenal lymphangioma was diagnosed on histology and confirmed with immunohistochemical stains. One month after the surgery the ureteral stent was removed. The patient was free of disease after a 3-month follow-up pe-riod. We report the case and discuss the management of perirenal lymphangiomatosis with a literature review.