Moyamoya syndrome associated with neurofibromatosis type 1 in a pediatric patient

Neurofibromatosis type 1 is a multisystem genetic disease of autosomal dominant transmission that reveals important cutaneous manifestations such as café-au-lait spots,multiple neurofibromas,and ephelides in skin fold areas,as well as hamartomatous lesions in the eyes,bones,glands,and central nervous system. moyamoya disease is a rare progressive vaso-occlusive disorder that occurs with important ischemic cerebrovascular events. despite the rarity of this association in childhood,children diagnosed with neurofibromatosis type 1 and focal neurologic symptoms should be investigated for moyamoya syndrome. the present study reports the case of a pediatric patient with a rapidly progressive cerebrovascular accident and a late diagnosis of neurofibromatosis type 1 associated with moyamoya disease. © 2017 by anais brasileiros de dermatologia.