Abstract： Objective To investigate the clinical features,diagnosis,therapeutic strategy and prognosis of Primary Intracranial Choriocarcinoma ( PICCC ).Methods A retrospective review of four patients with PICCC diagnosed histopathologically was performed during May 2002 to May 2010,and all patients were followed up and pertinent literatures were reviewed.Results There were four young male children studied.The average age of all patients was 13.75 years.Everyone had a mass lesion in pineal region where its average diameter was 4 ～ 5 cm.Three cases were given preoperative diagnostic irradiation,of which two cases had hemorrhage after irradiation.All received operation with trans - corpuscallosum -fornix approach,three cases received total resection,and the other one had subtotal resection.After operation,all cases were supposed to have chemotherapy.3 patients died finally during a follow - up period range from 1 month to 5 years with chemotherapy,and the mean survival time was 31.7 months.Conclusions PICCC are extremely rare and the most malignant of primary intracranial GCTs,patients have poor prognosis.Markedly elevated β-HCG levels in serum or cerebrospinal fluid are strongly suggested to be found with PICCC while mild elevations can be seen with other GCTs.The authors recommend surgery for patients with PICCC combined with chemotherapy.Radiotherapy is not the conventional therapy due to the increasing risk of hemorrhage.