PCARE (photoreceptor cilium actin regulator) is an actin-associated protein essential for photoreceptor outer segment (OS) disk formation and maintenance 1. Structurally, PCARE contains a helical coiled coil domain, conserved binding sites for the kinase MAK, and EVH1 domain-binding linear motifs that are critical for its function 2. Functionally, PCARE recruits and activates the Arp2/3 complex activator WASF3 to the ciliary tip, driving actin polymerization and membrane expansion at the base of the photoreceptor OS where new disk formation initiates 1. Loss of PCARE or expression of disease-associated mutations impairs this actin-driven ciliary expansion process 1. PCARE mutations cause severe inherited retinal dystrophies, including retinitis pigmentosa 54 (RP54) and cone-rod dystrophy 23 3. PCARE-associated retinopathy presents as severe generalized photoreceptor dystrophy with macular atrophy in all affected individuals 3. Clinically, patients experience progressive vision loss, with legal blindness occurring at a median age of 57 years, though foveal sparing often preserves central vision into late adulthood 3. The optimal therapeutic window for intervention appears to be before the fifth decade of life 3. Novel PCARE variants continue to expand the phenotypic spectrum, including bilateral macular colobomata 4.