A 12-year-old boy presented with vision loss since infancy and childhood fractures. Axial T2-weighted image (Fig. 1a) demonstrates small right globe with a retrolental mass (black arrow). The canal of Cloquet (arrowhead) ends at the level of the retrolental mass. Features are in keeping with posterior persistent hyperplastic primary vitreous (PPHV). The left side reveals severe microphthalmia with misshapen globe in keeping with phthisis bulbi (curved arrow) with associated mildly hypoplastic optic nerve (open arrow). Lumbosacral spine radiograph (Fig. 1b) shows osteoporosis, “picture framing,” biconcave compression deformities of vertebrae (black dashed arrows), and associated disc ballooning. Findings are diagnostic of osteoporosis-pseudoglioma (OPPG) syndrome with confirmed mutation in low-density lipoprotein receptor–related protein 5 (LRP5) gene. OPPG, a rare autosomal recessive disorder, presents with eye phenotype from defective vascularization ranging from milder vitreoretinal changes to congenital phthisis bulbi. Osteoporosis manifests with vertebral compression fractures, long bone fractures, and reduced bone mineral density.