Papillary cystadenoma of the epididymis is an occasional finding in men with von Hippel-Lindau disease. To date, an analogous lesion has not been described in women with the syndrome. We report a case of an asymptomatic papillary cystadenoma of the mesosalpinx found incidentally during surgery for a renal cell carcinoma in a woman with von Hippel-Lindau disease. This finding supports the notion that similar lesions may arise in both men and women with von Hippel-Lindau disease, perhaps from embryologically related portions of the urogenital tract.