Neurofibromatosis (Von Recklinghausen disease) is a genetically transmitted, multisystemic disorder characterized by abnormalities of the skin, peripheral nerves, bone, and soft tissue. Symptoms appear early in life and vary with the type and severity of the disease. Care of patients with neurofibromatosis (NF) presents a complex challenge for nursing, the multidisciplinary team, and the family. This article profiles the progression of neurofibromatosis in one girl from birth through adolescence. The author emphasizes that while health care workers ably provide expertise and assistance, the ultimate responsiblity for decision making rests with the child and her family.