WDR19 (WD repeat domain 19) is a ciliary protein essential for intraflagellar transport (IFT) complex A assembly and retrograde ciliary transport 1. As a component of IFT-A, WDR19 mediates GPCR entry into cilia and is critical for cilium function and assembly 1. The protein contains six WD repeats, a clathrin heavy-chain repeat, and three transmembrane domains, with conserved expression from C. elegans to humans 2. Biallelic WDR19 variants cause multiple ciliopathies including nephronophthisis-13, Senior-Loken syndrome-8, short-rib thoracic dysplasia-5, and cranioectodermal dysplasia-4 3. Notably, WDR19 mutations have emerged as an unexpected cause of retinal degeneration phenocopying Stargardt disease 456. WDR19-associated retinopathy presents heterogeneously with features including nyctalopia, cone-rod degeneration patterns, photoreceptor atrophy, and notably preserved foveal regions in some patients 56. While nephronophthisis typically develops later, patients with CEP290 or IQCB1 variants develop earlier retinopathy onset 7. Pancreatic involvement is rare in WDR19-related ciliopathies but has been documented 8. These findings underscore WDR19's pivotal role in photoreceptor and renal ciliary function, with significant implications for genetic diagnosis of inherited retinal and kidney diseases.