Objectives: To report the optical coherence tomography (OCT) fndings in patients with Wolfram syndrome. Materials and Methods: Four patients who fulfilled the criteria for Wolfram syndrome were recruited to the study. In all patients, OCT was performed with Stratus OCT (OCT-3, Carl Zeiss Meditec, Inc. Germany). The fast retinal nerve fiber layer (RNFL) and fast macular thickness protocols were used to measure the RNFL and macular thickness, respectively. The fast optic disc protocol was used to determine the cup-to-disc ratios of the optic disc. All patients were examined with VEP (Retimax, CSO Strumenti Oftalmici, Florence, Italy). Results: In eight eyes of four patients (3 male and 1 female) with a mean age of 18.5±2.08 years (range 16-21 years), RNFL, macular thickness, and cup-to-disc ratios were determined. The mean RNFL was 42.2±5.6 μm (range 34.1-49.5 μm), while the mean macular thickness and cup-to-disc ratios were 145±15 μm (range 125-160 μm) and 0.79±0.07 (range 0.7-0.92), respectively. There was a moderate negative correlation between VEP latencies and macular and RNFL thicknesses (Spearman correlation coefficient was -0.23 and -0.34, respectively). Conclusions: RNFL loss and secondary optical atrophy are severe complications that may affect the visual acuity in patients with Wolfram syndrome. Retinal changes in these patients may be quantified and can be observed using OCT.