Malignant peripheral nerve sheath tumor (MPNST) is a malignant spindle cell tumor of the soft tissue thought to be derived from the components of nerve sheath. MPNSTs are mainly located in the buttocks, thighs, brachial plexus, and paraspinal region. The objective of this article is to describe a case of neurofibromatosis type 1 who developed neurofibrosarcoma of the right lateral thoracic nerve with thoracic meningoceles, a rare coincidental finding which has not yet been reported in the English medical literature, and how both the conditions were managed in the same sitting.