Von Recklinghausen's disease or type 1 neurofibromatosis (NF-1) is an autosomal dominant condition with a varied clinical expression. Disease manifestations may range from café au lait spots of the skin to severe cosmetic and functional complications that affect bone and nervous tissues. Oral manifestations of NF-1 are common, affecting approximately 72% of patients. Treatment of neurofibromas is surgical resection to solve esthetic or functional problems. The aim of this paper is to report an interesting case of NF-1 with cutaneous lesion of upper lip and middle finger of left hand along with generalized lesions on the entire body.