视网膜色素变性可合并眼部其它异常,文献报道有合并视乳头星形细胞错构瘤、视乳头玻璃疣、假性视乳头水肿。就本文作者所知,合并视乳头水肿极为罕见。本文报道一例青年性视网膜色素变性合并单侧视乳头水肿。病例:女性,15岁,足月顺产,出生体重3350克。双亲非血族联姻。家族史无特殊。母亲在妊娠期无严重疾患。患者于4个月时有内隐斜。1岁时有内斜合并远视散光,瞳孔光反应灵敏,双侧视网膜色素呈斑驳样沉着,右眼视乳头轻微异常。给予配镜处理。2岁起有夜盲,此后家庭搬迁,失去随访。12岁时又返回我院复诊。当时矫正视力为右0.2,左0.1。眼压正常,内斜视及钟摆式眼球震颤。双眼角膜 Retinitis pigmentosa can be combined with other abnormalities in the eye, reported in the literature with papilledema astrocytoma, papilloma, pseudo papilloedema. As the authors of this article know, combined papilledema is extremely rare. This article reports a case of young retinitis pigmentosa with unilateral papilledema. Case: Female, 15 years old, term full-term birth, birth weight 3350 grams. Parents of non-kinship marriage. No special family history. Mother during pregnancy without serious illness. Patients had implicit tilt at 4 months. 1 year old with intracranial hyperopia astigmatism, pupil light sensitive, bilateral retinal pigment mottled like calm, mild abnormality of the right eye as the optic nerve. Giving glasses treatment. 2-year-old have a night blindness, since the relocation of families, lost follow-up. At 12 years of age and returned to our hospital referral. At that time, corrected visual acuity was right 0.2, left 0.1. Normal intraocular pressure, esotropia and pendular nystagmus. Eyes cornea