Retinitis pigmentosa (RP) is an inherited neurodegenerative disease characterized by progressive photoreceptor cell death and retinal pigment epithelium (RPE) atrophy, leading to vision loss. It is the most common inherited retinal dystrophy, with a global prevalence of 1/4000. RP is classified based on inheritance patterns, including autosomal dominant (adRP), autosomal recessive (arRP), and X-linked (x-RP), with varying disease progression and prognosis. Non-syndromic RP, the most common form, is caused by mutations in genes involved in retinal function, while syndromic RP involves systemic manifestations due to mutations in genes affecting multiple tissues. The disease presents with symptoms such as night blindness, peripheral vision loss, and visual field defects. Diagnosis involves clinical tests like electroretinography (ERG), optical coherence tomography (OCT), and fundus examination. ERG measures photoreceptor activity, while OCT evaluates retinal layer morphology. The pathomolecular mechanisms of RP are complex, involving genetic mutations, oxidative stress, mitochondrial dysfunction, retinal remodeling, and inflammation. Over 90 genes are associated with RP, with the RHO gene being the first identified. Mutations in RHO, PRPH2, and RPGR are among the most common causes of adRP, arRP, and x-RP, respectively. Therapeutic approaches for RP include antioxidant agents like vitamin A palmitate, hyperbaric oxygen therapy (HBO), stem cell therapy, and gene therapy. Vitamin A has shown some protective effects but is not curative. HBO has shown promise in improving ERG responses but is associated with potential side effects. Stem cell therapy, particularly using mesenchymal stem cells (MSCs), has shown safety and some efficacy in clinical trials. Gene therapy, especially using adeno-associated virus (AAV) vectors, has been successful in treating RPE65-related RP, with voretigene nepavorec approved by the FDA. Despite these advances, RP remains challenging to treat, with no curative options available. Gene therapy is the most promising approach, but its effectiveness is limited by the heterogeneity of mutations and the need for early intervention. Other emerging therapies, such as CRISPR/Cas9 gene editing and optogenetics, show potential but require further research. Stem cell therapy and HBO are still in early stages of clinical evaluation. Continued research is essential to develop effective, safe, and accessible treatments for RP.Retinitis pigmentosa (RP) is an inherited neurodegenerative disease characterized by progressive photoreceptor cell death and retinal pigment epithelium (RPE) atrophy, leading to vision loss. It is the most common inherited retinal dystrophy, with a global prevalence of 1/4000. RP is classified based on inheritance patterns, including autosomal dominant (adRP), autosomal recessive (arRP), and X-linked (x-RP), with varying disease progression and prognosis. Non-syndromic RP, the most common form, is caused by mutations in genes involved in retinal function, while syndromic RP involves systemic manifestations due to mutations in genes affecting multiple tissues. The disease presents with symptoms such as night blindness, peripheral vision loss, and visual field defects. Diagnosis involves clinical tests like electroretinography (ERG), optical coherence tomography (OCT), and fundus examination. ERG measures photoreceptor activity, while OCT evaluates retinal layer morphology. The pathomolecular mechanisms of RP are complex, involving genetic mutations, oxidative stress, mitochondrial dysfunction, retinal remodeling, and inflammation. Over 90 genes are associated with RP, with the RHO gene being the first identified. Mutations in RHO, PRPH2, and RPGR are among the most common causes of adRP, arRP, and x-RP, respectively. Therapeutic approaches for RP include antioxidant agents like vitamin A palmitate, hyperbaric oxygen therapy (HBO), stem cell therapy, and gene therapy. Vitamin A has shown some protective effects but is not curative. HBO has shown promise in improving ERG responses but is associated with potential side effects. Stem cell therapy, particularly using mesenchymal stem cells (MSCs), has shown safety and some efficacy in clinical trials. Gene therapy, especially using adeno-associated virus (AAV) vectors, has been successful in treating RPE65-related RP, with voretigene nepavorec approved by the FDA. Despite these advances, RP remains challenging to treat, with no curative options available. Gene therapy is the most promising approach, but its effectiveness is limited by the heterogeneity of mutations and the need for early intervention. Other emerging therapies, such as CRISPR/Cas9 gene editing and optogenetics, show potential but require further research. Stem cell therapy and HBO are still in early stages of clinical evaluation. Continued research is essential to develop effective, safe, and accessible treatments for RP.