Retinitis pigmentosa (RP) is the most prevalent form of hereditary retinal dystrophy. Typically, the disease is bilateral and symmetrical. Cases of unilateral RP are very rare and only isolated cases or short series have been reported. We present two cases of unilateral RP. In the first, a 38-year-old woman was diagnosed with unilateral RP in the left eye, and followed up for 10 years. In the second, we describe a 64-year-old male with unilateral RP complicated with cystoid macular edema in the right eye, from the time of diagnosis, followed up for 6 years.