Objective：To investigate the role of chemotherapy in the diagnosis and treatment of children with intracranial gonioma. Methods：Cases to be diagnosed as intracranial gonioma in the Children’s Hospital of Chongqing Medical University from January 2010 to December 2013 were divided into intracranial gonioma and non-intracranial gonioma according to tumor markers alpha-fetoprotin（AFP） and human chorionic gonadotropin beta（β-HCG） test results. Cases in intracranial gonioma group were treated by CE chemotherapy for two courses，and the curative effect including partial remission（PR） or complete remission（CR） was evaluated using solid tumor evaluation criteria-response evaluation criteria in solid tumors（RECIST）. After making a definite diagnosis，patients over 3 years old carried out radiation therapy and follow-up chemotherapy and patients less than 3 years old were administrated with chemotherapy until over the age of 3 years，and then received extended radiotherapy. Non-intracranial gonioma cases were administered with BCE or ICE chemotherapy combined with full dose of central nervous system radiotherapy. Whether carrying out the second surgery was decided according to the actual conditions. Results：Twenty-five patients were followed up，19 patients（95%） out of 20 suspected patients with intracranial gonioma were PR after receiving chemotherapy. After being diagnosed as intracranial gonioma，15 patients（100%） in over than 3-year-old group were CR after chemotherapy，with 100% survival rate and an average follow-up period of 2 years. In less than 3-year-old group，one patients experienced relapse and craniospinal metastasis after six courses of chemotherapy and abandoned the treatment，the other 2 patients were successfully completed deferred radiotherapy after six courses of chemotherapy，both CR until now. During the current follow-up，one patient with stable tumor was diagnosed as teratomas by pathologic examination excluding the diagnosis of intracranial gonioma and underwent resection. There were 5 cases of non-intracranial gonioma including 2 cases of yolk sac tumor and 3 cases of mixed intracranial gonioma. One patient with yolk sac tumor died during the chemotherapy and one patient lived with event free survival for 2 years after successfully completing the resection and the chemotherapy. Three patients with mixed intracranial gonioma received comprehensive treatment of chemotherapy plus radiotherapy plus chemotherapy and lived with event free survival for 1-3 years. Conclusions：Diagnostic chemotherapy is effective since intracranial gonioma is very sensitive to the CE chemotherapy. But taking into consideration of higher recurrence rate after chemotherapy，diagnostic chemotherapy must combine with radiotherapy. Chemotherapy combined with radiotherapy（reducing the effective dose and scope） can improve the survival and quality of life. Patents with age less than 3 years old are still the difficulties in the treatment and research. Chemotherapy treatment is effective for treating non intracranial gonioma and chemotherapy combined with radiotherapy is the main therpay.