Neurofibromatosis type 1 (NF1) (von Recklinghausen disease) is an autosomal dominantly inherited neurocutaneous disorder which affects many systems like ocular, cutaneous and nervous systems and seen in 1:3500 births. Cardinal diagnostic criteria of NF1 were established in 1987 by National Institutes of Health Consensus.Early diagnosis and the findings of NF1 are unclear in childhood, but with age the clinical symptoms become apparent. NF1 is occasionally associated with mental retardation.In this report, together a review of the literature, we present a quite elderly patient, 79-year-old-man, with NF1 suffering from metastatic malignant peripheral nerve sheath tumor in the axillary lymph node invading the brachial plexus and pleura. Moreover, this enormous metastatic mass had restricted movement of the extremity. He had multiple neurofibromas of different sizes almost covering his entire body, massively. To the best of our knowledge, our patient's malignant peripheral nerve sheath tumor and massive neurofibromatosis is a rare case to present in the eighth decade of life.