Abstract Background Sporadic Creutzfeldt-Jakob Disease (sCJD) is a rare neurodegenerative prion disease that presents with symptoms of rapid neuropsychiatric decline including dementia, behavioural abnormalities and loss of higher cortical function. Patients commonly present with rapidly-progressive neuromotor symptoms such as ataxia and myoclonus. Very few cases of CJD have been reported in which the patient initially presents with stroke symptoms such as hemiparesis as their primary presenting symptom. We present a case of a 56 year old male who initially presented to the stroke unit with waxing and waning left sided weakness and a non-corresponding ipsilateral left-sided acute parietal infarct on diffusion-weighted MRI. Over four weeks, his condition progressively worsened with declining cognitive function, motor dysfunction, sphincter dysfunction and eventual death. Methods Methods used to confirm the suspected diagnosis included: (1) Periodic sharp wave complexes are the most classical EEG finding for CJD and are seen in two third of the patients diagnosed with sporadic CJD, (2) The presence of the 14–3-3 protein in CSF has been found to be a sensitive and specific marker for sporadic CJD, (3) Neuroimaging plays a vital role in the diagnosis of CJD including serial MRIs and EEG sufficient to exclude other differential diagnoses. Results MRI brain revealed an acute cortical infarct within left parietal lobe measuring 3 mm.A n EEG demonstrated periodic sharp wave complexes occurring maximally in the left centro-temporal region. EEG finding was suggestive of a diagnosis of a prion disease. CJD Surveillance Unit confirmed the cerebrospinal fluid analysis was positive for RT-QuIC indicating sporadic CJD. Conclusion In cases of patients presenting with clinical signs and symptoms of a stroke with subsequent ipsilateral neurological findings on MRI, CJD should be included as a differential diagnosis. Additionally, a lumbar puncture, not routinely recommended in stroke patients, should be performed early to aid in early detection of CJD in order to confirm diagnosis and optimise the ceiling of care.