We report a rare case of an elderly woman presented with right renal mass with invasion of renal vein and several small lymphadenopathy in the hilar area. The diagnosis of kidney cancer is suspected and the patient underwent open radical nephrectomy, surrenalectomy and lymphadenectomy dissection. The pathologic examinations find a rosai dorfman disease. This unusual benign entity is uncommon in the kidney, but in medical imaging, it may simulate an infiltrative renal neoplasm, especially a lymphoma or leukemia or even renal cell carcinoma. A comprehensive literature review was undertaken to summarize the clinical and pathologic features of this disorder.