The authors present a case of permanent visual loss secondary to occipital lobe calcification in coeliac disease, the first reported such case. The 58-year-old man had been diagnosed with coeliac disease 20 years prior to his visual investigations and had a 40 year history of poor vision that had never been fully investigated. He was found to have significantly reduced visual acuity of less than 6/60 bilaterally and a right homonymous inferior quadrantanopia. His optic discs appeared to be normal, however CT and MRI scans revealed bilateral occipital calcification. The mechanisms for visual loss from coeliac disease are largely unknown, the authors suggest that a major cause is genetic and immunological, leading to inflammatory changes resulting in cerebral calcification and damage to retrochiasmal CNS tissue.