Neurofibromatosis type 1 is a genetic disorder that follows an autosomal dominant pattern of inheritance. Ocular involvement is not uncommon, but spontaneous dialysis of the retina in the absence of a history of trauma is a rare clinical entity. Rare cases of retinal involvement such as retinal detachment or dialysis of ora serrata could be linked with the abnormal cell-matrix formation in neurofibromatosis type 1. Here, we present a case of a 36-year-old man having Neurofibromatosis Type 1 with spontaneous dialysis of ora serrata without prior history of ocular trauma. A routine fundoscopic examination should be done in addition to the examination of the anterior chamber in patients with neurofibromatosis type 1 despite the absence of ocular complaints.