The diagnosis of PHPV is often made difficult by its extremely broad array of clinical manifestations, its etiologic heterogeneity, and frequently opaque ocular media. The CT and MR findings are discussed in terms of how they can be of use in the differential diagnosis of PHPV. The strengths and weaknesses of each of these diagnostic methods is detailed in regard to patients who present with unilateral leucocoria, microphthalmos, lens opacity, and retinal detachment.