Splicing factors(SFs)are a type of protein that serves as an integral component of the dynamic spliceosome complex. The spliceosome, similar to “scissors”, has the ability to accurately process precursor RNA(pre-mRNA)in eukaryotes and generate a diverse range of mRNA sequences. This process is important for gene regulation and protein expression. Pre-mRNA processing factor 31(PRPF31)is a widely expressed SFs in various biological tissues. However, mutations in PRPF31 are specifically linked to the development of autosomal dominant retinitis pigmentosa(adRP), known as PRPF31-RP. Currently, the pathogenesis of PRPF31-RP is still unclear. This article reviews the research progress on the molecular mechanism of PRPF31 in the development of adRP and the progress in PRPF31-RP treatment from the perspective of tissue damage and impairment of biological processes caused by PRPF31 mutation or deletion, in order to provide new ideas on the pathogenesis and treatment of PRPF31-RP.