目的提高对男性颅内原发性绒毛膜癌的认识。方法对1例经开颅手术病理确诊为颅内原发性绒毛膜癌男性患者的临床资料进行分析,并结合国内外文献进行复习。结果男性颅内原发性绒毛膜癌是临床少见病例,恶性程度高,临床除颅内占位性病变外常无其他特异性表现,如患者不及时治疗可在短期内死亡。结论影像学发现青少年颅内占位性病变且有出血倾向,血或尿β-HCG升高的患者应考虑到该疾病,应尽早给予包括手术和放化疗为主的综合治疗。 Objective To improve the understanding of male primary choriocarcinoma. Methods The clinical data of 1 male patient with intracranial primary choriocarcinoma who had been diagnosed by craniotomy were analyzed and reviewed with references both at home and abroad. Results Male intracranial primary choriocarcinoma is a rare clinical case with high degree of malignancy. In clinical practice, there are no other specific manifestations besides intracranial space occupying lesions. If the patient is not treated promptly, it may die within a short period of time. Conclusions Radiographic findings in children with intracranial space-occupying lesions and bleeding tendency, elevated blood or urine β-HCG should be considered in patients with the disease and should be given as soon as possible, including surgery and radiotherapy and chemotherapy-based comprehensive treatment.