患者 ,男 ,16岁 ,因发作性意识丧失 4个月 ,右下肢无力 1月入院。查体 :额顶部有一 5cm× 5cm大小黑痣 ,整个背部上起颈部下至尾骨呈黑色改变 ,并有毛发生长。CT示左额顶占位 ,即行手术治疗 ,术中发现脑膜着色深 ,以中线区域黑变明显 ;术野内全部脑膜和脑实质弥漫性黑色素沉着 The patient, male, was 16 years old. He suffered a loss of paroxysmal consciousness for 4 months, and his right lower limb was unable to be admitted to hospital in January. Physical examination: There is a 5cm x 5cm black pimple at the top of the forehead. The entire back of the head is black from the neck to the coccyx and has hair growth. CT shows the left frontofrontal space occupying position, that is, surgical treatment, the deep coloration of the meninges was found during the operation, and the darkening of the midline region was obvious; diffuse melanin deposition in all meninges and brain parenchyma in the operative field.