Patient: 64 years of age, male, BCVA RA:-5,00 -0,75|90°=0,25; LA: -5,25 -0,25|90°=0,4; IOP 20/20 mmHg General Medical History: arterial hypertension, cerebral infarction in 2006, systemic therapy with marcumar, level of ornithin 114 µmol/l (normal range 39-96 µmol/l ) Ocular Medical History: myopia. Main Complaint: slow deterioration of reading. Methods: Colour Photography, FFA, OCT, ERG. Findings: Colour photography: at OD & OS hypopigmented fundus, retinal pigment epithelium and choriocapillaris atrophy. FFA: at OD & OS lesions with large irregular and confluent patches of RPE/CC atrophy in the midperiphery and in the peripapillary and parapapillary areas. Optical coherence tomography: at OD & OS changes in the retinal pigment epithelium, thinned neural retina with impaired lamination, shortening of the photoreceptor inner and outer segments in areas corresponding to RPE/CC atrophy, the neural retina fairly preserved over retinal pigment epithelium and choriocapillaris atrophy. ERG: at OD & OS reduced Discussion: Choroideremia (CHM) is an X-linked retinal dystrophy belonging to the family of blinding disorders. It is characterized by progressive degeneration of the choriocapillaris, retinal pigment epithelium and photoreceptors. CHM is caused by mutations in the Rab Escort Protein 1 (REP-1) gene, which encodes a protein involved in vesicular trafficking (1). Complete deletion of the CHM gene causes severe choroideremia. Mura et al. (2) presented results of serial ERGs documenting progression first of rod and then of cone disease. Literature: (1) Coussa RG, Traboulsi EI. Choroideremia: a review of general findings and pathogenesis. Ophthalmic Genet. 2012 Jun;33(2):57-65. doi: 10.3109/13816810.2011.620056. Epub 2011 Oct 21. (2) Mura M, Sereda C, Jablonski MM, MacDonald IM, Iannaccone A. Clinical and functional findings in choroideremia due to complete deletion of the CHM gene. Arch Ophthalmol. 2007 Aug;125(8):1107-13.