源于颅骨的颅内软骨瘤或软骨肉瘤发病率约占颅内肿瘤0.1%～0.2%,两侧同时发生者更罕见。Maffucci’s综合征以多发性内生软骨瘤合并血管瘤病为特征。目前尚无颅内软骨肿瘤合并Maffucci’s综合征的病例报告。本文报道1例。患者,男,33岁。患有先天性多发性内生软骨瘤病,右耳进行性听力减退18个月。入院前出现右面部感觉减退,眩晕及运动失调。神经系统检查发现右侧三叉神经第一、二支皮肤感觉迟钝,右侧面神经麻痹,眼震,共济失调。右手因内生软骨瘤而畸形,同时有多发性皮下浅蓝色血管瘤。右手 The incidence of intracranial chondroma or chondrosarcoma originating from the skull accounts for approximately 0.1% to 0.2% of intracranial tumors, and it is even rarer on both sides. Maffucci’s syndrome is characterized by multiple endochondromas with angiomatosis. There is no case report of intracranial cartilage tumor with Maffucci’s syndrome. This article reported 1 case. Patient, male, 33 years old. Congenital multiple endochondroma disease, right ear progressive hearing loss for 18 months. Right facial sensation, dizziness and dyskinesia appeared before admission. Neurological examination revealed that the first and second branches of the right trigeminal nerve were dull, and the right facial nerve was numb, nystagmus, and ataxia. The right hand is malformed due to the endogenous chondroma and there are multiple subcutaneous blue hemangiomas. Right hand