It accounts for 5-8% of childhood cancers. The commonest site of involvement is head and neck. It occurs with increased frequency in patients with neurofibromatosis. The most common presenting feature is painless mass (occasionally it may be painful), other symptoms are caused by displacement or obstruction of normal structures. Treatment is based on the primary tumor location and disease stage which includes chemotherapy and surgery. Completely resected tumors have the best prognosis.