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We describe a woman in her 30s who presented with progressive cognitive decline and reflex focal seizures triggered by specific auditory stimuli. Serum autoimmune testing demonstrated markedly elevated anti-glutamic acid decarboxylase 65 (GAD65) antibody titres (>2000 IU/mL). A paraneoplastic work-up revealed acute myeloid leukaemia (AML). After comprehensive neurological and haematological evaluation, she was diagnosed with paraneoplastic anti-GAD65-mediated autoimmune epilepsy, consistent with limbic encephalitis. This case is notable as it highlights a rare association between AML and paraneoplastic limbic encephalitis with anti-GAD65 antibodies.