Von Hipple-Lindau (VHL) disease is a rare familial cancer syndrome that is dominantly inherited and pre-disposes affected individuals to developing various tumors, including hemangioblastoma of the retina and central nervous system, and multicentric renal cell carcinoma. We report two cases of VHL disease with bilateral renal cell carcinoma. Case 1: A 53-year-old woman was referred to our hospital because of bilateral kidney tumor incidentally found. We performed left laparoscopic radical nephrectomy and laparoscopic nephrectomy, ex vivo excision and reconstruction, and autotransplantation for the right kidney. Case 2: A 43-year-old woman was referred to our hospital because of left kidney tumor incidentally found. Because the suspectious lesion in the right kidney was very small, we decided to follow it up with no treatment. We performed laparoscopic nephrectomy, ex vivo excision and reconstruction, and autotransplantation for left kidney.