Sixty-two patients, 48 with a diagnosis of persistent hyperplastic primary vitreous (PHPV) and 14 with monocular congenital cataract (MCC) are reported. Nineteen patients with a diagnosis of PHPV and all patients with MCC received surgery with attempted visual rehabilitation. Early age of surgery, prompt optical correction with contact lens and aggressive patching therapy are required for successful visual rehabilitation. Glaucoma, retinal or optic nerve pathology, surgery later than three months of age or combinations of these factors were associated with poor prognosis. Isolated microphthalmos was not correlated with poor prognosis. Binocular fixation pattern was used to monitor patching therapy. Optokinetic nystagmus, visual evoked potentials or forced preferential looking were not employed. Of the 33 patients who underwent surgery, 10 (30%) achieved good (greater than or equal to 20/50) visual acuity, 8 (24%) achieved fair (20/60 to 20/100) and 15 (46%) achieved poor vision. In selected patients with the diagnosis of PHPV or MCC, visual rehabilitation can be achieved.