Germ cell tumor of testis in a patient with von Hippel-Lindau disease. Germ cell testicular tumor is a previously undescribed entity in association with von Hippel-Lindau disease. This case exemplifies the variety of pathologic entities encountered in von Hippel-Lindau disease and stresses the importance of thorough evaluation of the patient, as well as careful follow-up, to ensure early detection of potentially malignant lesions. 