Neurofibromatosis (NF) is a group of autosomal dominant disorders. Three clinically and genetically distinct forms have been recognized: neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and schwannomatosis. The hallmarks are the multiple café-au-lait macules and neurofibromas. Neurofibromas do not only occur in the neuraxis but may also be found in the oropharnyx and larynx. A case of NF1 along with autism spectrum disorder and tracheomalacia status post tracheostomy was described.