Von Recklinghausen's disease(V.R.D.), a tumor originating from the neuroectodermal and mesodermal(connective tissue) elements are included, may occur in the almost all regions of the body, but it is very rarely seen in the nasal alae. The disease is characterized by multiple Café-au-laits spots, generalized cutaneous neurofibromatosis, CNS tumors, skeletal abnormal, mental retardation, a myriad of associated somatic and endocrine abnormalities. The authors have experienced a case of V.R.D. on the left nasal alae in a 15-years-old boy. Under the general endotracheal anesthesia, the growing mass was removed with subcutaneous transsection, using incision of Hauberisser, and then he left alae and septum were reconstructed. This is the report of a case with review of literature.