The last male patient presented in our clinic with a severe retinal degeneration at the age of 50 years and was found to be homozygous for an in-frame insertion/deletion in PROM1. On the basis of patient history, clinical findings and genetic results, the clinical diagnosis was changed to autosomal recessive retinitis pigmentosa. The gene discussed is PROM1; the disease is autosomal recessive retinitis pigmentosa.