In this report a case of bilateral papillary cystadenoma of the epididymis is described, a tumor associated with the Von Hippel-Lindau's disease (cerebelloretinal hemangioblastomatosis). Reports about the Von Hippel-Lindau's disease do only infrequently mention epididymal adenomas because the latter is mostly not associated with any symptoms. Retinal angiomatosis, cerebellar hemagioblastoma, pheochromocytoma and renal cell carcinoma are the lesions in the Von Hippel-Lindau's disease which cause symptoms and as a consequence disease. Because of some histological similarities between papillary cystadenoma and the clear cell type of renal adenocarcinoma a metastasis of a renal cell carcinoma has to be added to the list of differential diagnosis in case of an epididymal benign tumor apart from adenomatoid tumor, fibroma, lipoma,... The epididymal papillary cystadenoma, which is the only known benign epididymal tumor of epithelial origin, is considered a hereditary hamartoma. The precise histogenetic origin is still controversial.