Neurofibromatosis or Von Recklinghausen's disease is an autosomal dominant disorder, associated with an excess of malignant tumors. The most common neurofibromatosis-associated malignancies are derived from neurogenic tissues, although several malignancies that did not originate from neurogenic tissue are also described. This paper provides the first documentation of a patient with neurofibromatosis and a mixed germ cell tumor of the testis.