There are several treatment modalities for Langerhans' cell histiocytosis (LCH) of bone, formerly usually referred to as eosinophilic granuloma, including surgery, radiotherapy and chemotherapy. In spinal lesions, surgery is not recommended generally, because of the potential for reconstitution of vertebral height. We report a case of multifocal monosystemic LCH treated by surgery and chemotherapy for calvarial and spinal lesions. A 7-year-old boy was initially operated for an occipital monostotic LCH of bone. Then, he was treated by surgery for cervical and thoracic lesions, and by chemotherapy with vinblastine. The patient had no complaints at the 24-month follow-up. Although surgery is not recommended for spinal lesions in children with LCH of bone, short segmental fusion and internal fixation may be a suitable choice for selected cases, especially in children who are not likely to comply with activity restrictions and in those who it is difficult to follow up. The treatment must be planned according to the characteristics of the patient. Copyright (C) 2003 S. KargerAG, Basel.