Creutzfeldt-Jakob disease (CJD) is a rare neurodegenerative disorder with characteristic clinical and diagnostic features. We describe the unusual case of an elderly man who presented to our ophthalmology clinic with horizontal diplopia secondary to an abducens nerve (cranial nerve six) palsy and was subsequently diagnosed with CJD. Given the non-treatable nature of this disease, ophthalmologic management goals included symptomatic relief and quality of life improvement. Precautions related to the ophthalmologic management of CJD have also been addressed in this case report.