Objective:To explore the clinical classifications and treatments of craniocerebral Langerhans cell histiocytosis (LCH).Methods:Patients with histopathologically conformed LCH in our hospital from January 2008 to January 2019 were classified into 7 types according to the International Histocell Association clinical typing: type I (single site of skull), type II (single site of skull and involvement of central nervous system [CNS]), type III (multiple sites of skull), type IV (single site of CNS), type V (multiple sites of CNS), type VI (low-risk multiple sites of multisystem), and type VII (high-risk multiple sites of multisystem). According to the classification, two treatment methods were adopted. Group A (patients with type I) was treated with local resection, and Group B (patients with type II, III, IV, VI, and VII) was treated with chemotherapy based on Japanese Langerhans Cell Histiocytosis Study Group (JLSG) protocol after local resection or biopsy; according to the efficacy standards of JLSG, the treatment efficacies were evaluated.Results:The study covered 17 patients from Group A (type I) and 29 patients from Group B, including 2 with type II, one with type III, one with type IV, 10 with type VI, and 15 with type VII. During the follow-up period of one-10 years, there was no recurrence in 17 patients from group A; 27 out of 29 patients from group B had effective induction in postoperative JLSG chemotherapy, enjoying induction efficiency of 93.1%; two patients were treated with other chemotherapy regimes, and one of them was followed up for one year and his condition was stable.Conclusion:For type I craniocerebral LCH patients, clinical cure can be obtained with local resection; for type II, III, IV, VI, and VII LCH patients, good curative effect can be obtained with JLSG chemotherapy after local resection or biopsy; for LCH patients who have no reaction or disease progress in the first 6 weeks of induction, the curative effect can be improved by changing the rescue plan in time.