Megalocornea and persistent hyperplastic primary vitreous masquerading as congenital glaucoma.

A 7-month-old infant referred with suspected bilateral congenital glaucoma was noted under general anaesthesia to have simple megalocornea in association with contralateral glaucoma in an eye suspected of harbouring an intraocular tumour. Following enucleation, histopathology demonstrated persistent hyperplastic primary vitreous. The presence of simple megalocornea of autosomal recessive inheritance in association with contralateral persistent hyperplastic primary vitreous in a buphthalmic eye is of ophthalmic interest, and appears to be unique.