Objective: To describe and analyze the clinical features and results of exome sequencing of a distinct form of retinitis pigmentosa (RP) associated with retinal vascular occlusion (RVO). Design: This case series was a retrospective study. Participants: 23 patients with RP/RVO during 1992-2015. Methods: Information obtained from medical records includes age, gender, clinical history, visual acuity, and results of investigations using the slit-lamp, indirect ophthalmoscope, fundus photography, optical coherence tomography, angiography, electroretinography (ERG) and visual evoked potentials (VEP). Exome sequencing was performed in 8 blood samples. Results: Both eyes were affected. The optic disc was pale in 25 eyes, pale-white in 20 and normal in 1. Retinal vessels were attenuated in all eyes. There were widespread retinal pigment epithelium (RPE) abnormalities, including pigment discoloration and pigmented spots; no 'bone spicules' were observed. There was no inflammation, exudation, hemorrhage, neovascularization or proliferative vitreoretinopathy. Dye filling of vessels (angiography) was minimal/absent in 22 eyes, extended 1-3 diameters from the disc in 12, reached the posterior pole in 8, and reached the periphery in 4. ERG revealed severe a- and b-wave attenuation in 13 cases. VEP recordings showed implicit time prolongation and reduced amplitude. In 2 cases, progression from an intermediate to an advanced stage took 3.5-4 years. Prognosis was poor. In the results of exome sequencing, we found the SNP related RP, which had been reported previously. Conclusion: RP with RVO may be a distinct form of RP.