Tumours originating in peripheric nerves usually appear in patients with neurofibromatosis (NF) signs, presenting frequent combinations of tumours in nerves and cutaneous lesions. Nevertheless, this association is very rare in cases without NF. Therefore, the aim of the present article is to present a case of malignant melanoma and malignant schwannoma, without any described NF. A 69-year-old woman with antecedents of malignant melanoma diagnosed two years previously in the dorsum of the fifth finger of the left hand, treated by means of amputation of the finger. The patient presented a malignant epithelioid schwannoma adhered to the median nerve that required elbow amputation. One month later lung metastases appeared and the patient died. This case presented no known NF sign. However, a relationship must be searched for in the common origin of melanoma and schwannoma from the embryonic neural crest.