The low-density lipoprotein receptor-related protein 5 (LRP5; OMIM 603506 [1]) was first implicated in skeletal pathology in 2001 when it was determined that autosomal recessive loss-of-function pathogenic variants in LRP5, the gene encoding for the LRP5 receptor, leads to osteoporosis-pseudoglioma syndrome (OPPG; OMIM 259770 [2]), a disorder characterized by congenital or infancy-onset vision loss and severe osteoporosis [3]. Here, LRP5 is linked to osteoporosis-pseudoglioma syndrome.