About a case of Von Hippel-Lindau syndrome with association of retinal angioma, bilateral pheochromocytomas, bilateral renal cysts, the authors had many difficulties to confirm associated unilateral renal cell carcinoma. Three solid left renal tumors were not seen neither with abdominal ultrasound, nor with CT scan, but only with hypervascularity on renal angiography. Even intra-operative histologic examination fail to confirm the malignity. After the complete histologic examination of the tumorectomy piece, the unilateral renal cell carcinoma was secondary managed by radical nephrectomy, although the risk of bilateral carcinoma is known in this disease.