Eleven patients in this study are homozygous for the p.Arg245* variants while two siblings carry compound heterozygous variants, ¥p.Arg245*/p.Arg929*. Visual acuity assessments consistently show a decline between the third and fourth decade of life. Visual field loss in these subjects, shown in Table 1, further support severe retinitis pigmentosa. Macular atrophy and PSC cataract appear early and may contribute to the observed reduction in visual acuity. Optic nerve head pallor is also frequent and advanced. Normal Goldmann Visual Field Perimetry horizontal diameters are in the range of: V4e—150–160°, I4e—130–140°, and I1e—20–30°. BCVA: Best-Corrected Visual Acuity, OD: right eye, OS: left eye, HM: Hand Motion visual acuity, LP: Light Perception, HVF: Humphrey Visual Field, PSC: Posterior Subcapsular Cataract, NS: Nuclear Sclerosis Cataract, IOL: Intraocular lens, CME: Cystoid Macular Edema, ERM: Epiretinal Membrane, RPE: Retinal Pigment Epithelium, ONH: Optic Nerve Head, ND: Not Done, N/A: Not Available.