CEP83 (centrosomal protein 83) is a critical component of the centriolar distal appendages essential for primary cilium assembly 1. As part of the CEP83-SCLT1 protein module, CEP83 functions in the hierarchical recruitment of distal appendage proteins and regulates multiple steps of ciliogenesis, including preciliary vesicle recruitment, intraflagellar transport initiation, and CP110 removal 1. CEP83 phosphorylation by TTBK2 kinase is necessary for ciliary vesicle docking and cilia initiation 2. Beyond ciliary assembly, CEP83 serves as a mother centrosome marker and coordinates centrosome asymmetry with polarized endosome trafficking in radial glia progenitors, influencing cell fate decisions 3. CEP83 also regulates differentiation of human pluripotent stem cells toward kidney lineage, with loss-of-function disrupting intermediate mesoderm specification 4. Biallelic CEP83 mutations cause nephronophthisis 18 with variable phenotypes depending on mutation type 5. Loss-of-function variants produce elongated primary cilia and cause Oro-Facial-Digital syndrome combined with Joubert syndrome 6, while partial loss-of-function mutations cause infantile nephronophthisis with intellectual disability and central nervous system abnormalities 5. Recently, CEP83 mutations have been associated with bilateral perisylvian polymicrogyria, expanding the ciliopathy phenotype spectrum 7. These diverse clinical manifestations reflect CEP83's critical roles in ciliogenesis, centrosome organization, and developmental cell fate determination.