Von Hippel-Lindau disease is an autosomal dominant disorder occurring in 1 of 36,000 births and associated with various tumors and cysts in the central nervous system and other visceral organs. At present, metastasis from renal cell carcinoma (RCC) and neurologic complications are the most common causes of death from Von Hippel-Lindau disease. We report a case of Von Hippel-Lindau disease diagnosed during a screening and was found to have metastasis of RCC to a focal nodular hyperplasia lesion on the liver. In the literature, misdiagnosis of benign liver lesions as metastases of RCC has been reported, but there has not been a case reported to have a metastasis of RCC within a benign liver lesion. To our knowledge, this is the first case of RCC metastasis to a benign lesion of the liver.