A 75-year-old patient underwent ophthalmic surgery involving the retina to correct visual impairment. Six weeks later, he developed a tremor and a degree of cognitive impairment. Over the following weeks the symptoms progressed. A probable diagnosis of Creutzfeldt-Jakob disease (CJD) was eventually made based on the symptom complex and laboratory investigation. Evidence from his case notes was consistent with symptoms of CJD prior to the operation. It was recognized that there was a risk that reusable surgical instruments could have become contaminated and that there was a possibility of onward transmission of prion disease to other surgical patients.