This paper presents a case of biochemically proven congenital erythropoietic porphyria . the major ophthalmic manifestations are a dry eye and scleromalacia perforans . the implications of these are discussed 摘要本篇报告一经生化证明之先天性红血球性坡菲林病之病例。其主要之眼部表徵为乾眼和穿孔性巩膜软化,其临床症候之意义亦在文中予以讨论。