A 38-year-old man who had been followed with diagnosis of dilated cardiomyopathy and retinal angioma was referred to our hospital because of incidentally detected bilateral adrenal masses. Although he was normotensive, levels of catecholamine in urine were elevated and I131-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Screening of central nervous system by MRI revealed cerebellar hemangioblastoma. Right adrenalectomy and left partial adrenalectomy were performed, both of which tumors pathologically diagnosed as pheochromocytoma, followed by resection of the cerebellar hemangioblastoma. Five months later, abdominal CT revealed a left renal tumor and underwent left partial nephrectomy, being diagnosed as renal cell carcinoma. A right renal tumor was detected on follow up CT at 1 year after the partial nephrectomy. Since cardiac function was deteriorated, we have followed with careful observation. This is the second documented case of bilateral renal cell carcinomas and bilateral pheochromocytomas with VHL in Japan.