We experienced a rare case of primary intracranial choriocarcinoma treated successfully with synchronous chemotherapy and radiotherapy followed by three consecutive courses of chemotherapy without surgery. A 19-year-old male patient presented with a two-week history of diplopia, headache, nausea and vomiting. Neurological examination revealed visual disturbance, bilateral hearing loss, bilateral sixth nerve palsy, left skew deviation, downgaze limitation and clockwise torsional nystagmus on the left upgaze. During image evaluation, the patient suddenly presented with a confused mental status, bradycardia and decreased respiration. An emergency third ventriculostomy was conducted, followed by synchronous ifosfamide, cisplatin and etoposide chemotherapy (ICE; I, 900 mg/m(2); C, 20 mg/m(2); and E, 60 mg/m(2)) on days 1-5, and external radiotherapy (whole brain, 30 Gy/15 Fxs; local boost, 30 Gy/15 Fxs) followed again by three consecutive courses of chemotherapy. This therapy resulted in tumor regression by 63% and full improvement in the patient's neurological condition. However, it was difficult to remove the remaining tumor using a surgical approach due to the risk of postoperative hemorrhage and limited access. We planned to observe the remaining mass without surgery. This patient is now alive and the mass size has not changed for 18 months since treatment. This is the first report of the effectiveness of synchronous chemotherapy and radiotherapy followed by consecutive chemotherapy without surgery in a patient with primary intracranial choriocarcinoma.