Persistent hyperplastic primary vitreous (PHPV) occurs sporadically and few familial occurrences have been reported. The authors report PHPV unassociated with other congenital anomalies in male twins. One underwent a lensectomy for the management of angle-closure glaucoma and the other was treated for amblyopia. These cases provide further evidence to suggest autosomal recessive inheritance, though the possibility of developmental error cannot be excluded.