RESUMO
INTRODUCTION: Cystic lymphangioma is a rare benign vascular tumor that may arise in various sites, revealed at any age. Abdominal locations represent less than 10% of the cases preferentially involving the mesentery. We report a case of primary lymphangioma arising from the kidney. PRESENTATION OF CASE: A 50-year-old woman was admitted for severe left lumbar pain. Abdominal ultrasonography and computed tomography revealed a 6×10 cm multiloculated cystic mass with hydronephrosis, extending along the left renal hilum. Surgical exploration revealed a retroperitoneal cystic tumor. Anatomopathologic examination concluded it to be a cystic lymphangioma. DISCUSSION: The cystic lymphangioma is a benign malformative tumor of the lymphatic system. Surgery is the best curative treatment with complete excision, the prognosis is excellent. CONCLUSION: Primary renal lymphangioma is exceedingly rare. Medical imaging has certain limits for the diagnosis which required histological confirmation. The treatment of choice is surgical.