This original article illustrates the case of an 18-year-old woman who developed progressive supranuclear palsy-like syndrome following an uneventful aortic aneurysm repair. The patient was unaffected for the first 48 hours after surgery and then developed dysarthria, dysphagia (requiring a gastric tube), emotional lability and ophthalmoplegia. The authors have provided a video of the patient to illustrate how profound these deficits were. The patient underwent extensive contrasted fat suppressed cranial and orbital MRI, MRI tractography and functional MRI. Given the extensive degree of neurological impairment, the authors expected this advanced neuroimaging to be abnormal, however, it revealed no clear radiographic involvement, except for a tiny hypoechoic midbrain dot which is not considered sufficient to account for the patient’s deficits. This case attests to the unknown and unusual nature of this rare and devastating syndrome.