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Neuronal intranuclear inclusion disease (NIID) is a genetic leukodystrophy which mimics chronic small vessel disease and may be as common as CADASIL in individuals of certain ancestries. Thecondition can present with episodic encephalopathy and stroke-like episodes. It can be challenging to delineate between a common neurological presentation and the less common NIID.Method and Results We describe a case of an 82 year old female of East Asian descent who had several discrete episodes over 9 years of self-limited global aphasia and confusion attributed to delirium from urinary tract infections. During her most recent admission, she had persistent aphasia. MRI brain showed left temporal lobe swelling mimicking encephalitis, but also demonstrated the characteristic finding in NIID of abnormal diffusion restriction in the corticomedullary junction and T2 hyperintense signal in the splenium and paravermal white matter, which had also been present but not reported on historical scans. A skin biopsy demonstrated p62 and ubiquitin positive inclusionsconfirming the diagnosis of NIID.Conclusion This case highlights the challenges of differentiating common neurological conditions from rarer causes and detailed review of previous investigations, and aims to raise awareness of an uncommon but not rare condition.