ABSTRACT
Persistent hyperplastic primary vitreous(PHPV) is a congnital abnormality of the embryonic intraocular vasculature, resulting from the failure of regression of the primary vitreous in a full-term infant. PHPV is usually unilateral and characterized by leukocoria, elongated ciliary process, microphthalmia, cataract, rubeosis iridis, shallow anterior chamber, and secondary glaucoma. PHPV finally develops into retinal detachment, intractable glaucoma, and pthisis bulbi. Surgical treatment, involving lensectomy and vitrectomy, is not successful and poor visual result is obtained due to retinal abnormalities. A one-month-old girl was found to have persistent hyperplastic primary vitreous in both eyes, and she expired of unknown etiology at home 2 months after diagnosls.