We report a case of bilateral papillary cystadenoma of the epididymis thought to be a component of the von Hippel-Lindau syndrome. The patient was hospitalized initially because of infertility. He had undergone a craniotomy 2 years previously for the diagnosis of a cerebellar hemangioblastoma. Funduscopy had revealed angiomatous lesions of the left eye. The von Hippel-Lindau syndrome is discussed briefly and the importance of long-term urological followup for possible presentation of silent renal carcinoma is emphasized. Patients with bilateral epididymal papillary cystadenoma are prone to have other components of the von Hippel-Lindau syndrome, and clinical studies are indicated in all such patients.