Hereditary retinopathy (IRD) is a group of congenital retinal degenerative diseases caused by lesions in photoreceptor cells or retinal pigment epithelial cells. It has a high degree of genetic and clinical heterogeneity and is an important cause of incurable blindness in adolescents. Splicing variation is a special type of genetic variation caused by gene sequence mutations interfering with the normal RNA splicing process. It leads to abnormal rearrangement patterns of messenger ribonucleic acid during maturation, which is essentially different from the physiological isomers produced by normal selective splicing. IRD splicing variations mainly include classical site variations and deep intron site variations. Many breakthroughs have been made in targeted therapy for these variations. Antisense oligonucleotides, clustered regularly interspaced short palindromic repeats and associated protein 9 binding induced pluripotent stem cells, small molecule compounds, and spliceosome-mediated RNA trans-splicing techniques all show good application prospects. However, at present, related research is still in a fragmented state. A systematic review of splicing mutations and related treatment progress will provide important support for in-depth clarification of their pathogenic mechanisms and the development of effective therapies, bringing a glimmer of hope to such diseases that were once regarded as "incurable". Although there are still challenges such as low detection efficiency, poor treatment safety and individualized differences at present, with the continuous progress of molecular biology and gene therapy technology, it is gradually becoming possible to achieve precise diagnosis and radical individualized treatment of splicing mutation-related IRD, bringing hope to patients.