We determine the feasibility and safety of performing percutaneous cryoablation of renal tumors in patients with von Hippel-Lindau disease. We selected 2 men and 2 women with von Hippel-Lindau disease and radiographic determined solid renal tumors were selected to undergo percutaneous cryoablation. All patients underwent standard preoperative evaluation. An interventional magnetic resonance imaging unit was used for probe guidance and ice ball monitoring. The cryoablation procedure was performed with a 2 or 3 mm. cryoprobe using a pressurized argon gas system for ice ball formation. The patients were hospitalized overnight for observation and discharged home the following day. A followup computerized tomogram or magnetic resonance imaging scan was performed at 1 week, 1, 3, 6 and 12 months and every 6 months thereafter, along with physical examination, urinalysis, serum blood urea nitrogen and creatinine. A total of 5 tumors were treated ranging from 2.8 to 5.0 cm. in diameter. All patients underwent the procedure without difficulty with 2 requiring re-treatment due to residual tumor for a total of 7 treatments. At followup from 2 to 23 months there has been no radiographic evidence of recurrence at the cryoablated areas. Percutaneous cryoablation of renal tumors in patients with von Hippel-Lindau disease proved to be successful in this initial series. Although 2 patients had residual tumor after the initial cryoablation procedure re-treatment was performed with no adverse sequela. This minimally invasive therapy may allow patients with von Hippel-Lindau disease to avoid the necessity of multiple open surgical procedures.