Fifteen patients (16 eyes) with persistent hyperplastic primary vitreous (PHPV) were followed from one to four years. All but one of the nine eyes in the nonsurgical group showed progressive deterioration. A closed intraocular microsurgical (CIOM) approach posterior to the limbus was used in seven eyes. Three intraoperative complications involving the retina occurred because the retina in these cases inserted directly into the pars plicata and due to the presence of a thick, well-formed vitreous gel. Anterior placement of incisions is recommended to avoid intraoperative complications in small eyes. Early CIOM management is suggested in selected cases of PHPV to prevent progressive deterioration leading to phthisis and to afford reasonable cosmetic improvements.