报告一例罕见的经临床和病理组织学研究确诊为双眼原发性角膜脂肪变性症的病例。本例无家族眼病史，被随访观察１０年，双眼先后施行角膜移植术。右眼切除角膜片作病理组织学检查。光学显微镜和电子显微镜观察显示，角膜上皮和内皮基本正常，前弹力膜部分消失。角膜实质中层到深层，实质细胞内外存在多量大小不等的脂肪球样圆形物质和胆固醇结晶。实质深层有新生血管存在。未发现有全身的脂肪代谢异常 A case report of a rare case of clinically and histologically confirmed primary corneal steatosis in both eyes was reported. This case of non-family history of eye disease, was followed up for 10 years, the cornea has been implemented in both eyes. Right eye resection of corneal slices for histopathological examination. Light microscopy and electron microscopy showed that the corneal epithelium and endothelium were almost normal, and the anterior elastic membrane partially disappeared. The middle cornea to the middle of the deep, there are a large number of parenchyma cells inside and outside the size of fat-like round ball and cholesterol crystals. Substantial neovascularization exists. No systemic fat metabolism was found