Objectives: To report a rare case of renal lymphangioma in a 30-year-old female consulting with left flank pain.
Methods: We perform a bibliographic review and present the clinical, radiological and pathologic features.
Results: The patient underwent surgical treatment: radical nephrectomy. Final pathology report showed a renal lymphangioma.
Conclusions: Renal lymphangioma is a mesenchymal tumor of benign behavior which poses several diagnostic difficulties, both radiologically and histologically. This tumor may be confused with other renal tumors, including clear cell renal carcinoma.