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Dyke-Davidoff-Masson syndrome (DDMS) is a rare condition classically characterised by seizures with contralateral hemiparesis (often with facial asymmetry and variable cognitive impairment) and unilateral cerebral hemiatrophy on neuroimaging, frequently accompanied by compensatory calvarial thickening and paranasal sinus hyperpneumatisation. We report a woman in her early 20s with DDMS and medically refractory epilepsy who underwent a combined posterior quadrant disconnection with a selective posterior corpus callosotomy. Postoperatively, she experienced transient transcortical motor aphasia and hemiparesis, both of which resolved with rehabilitation. At 1 year, she remained free of generalised seizures and reported a marked reduction in drop attacks. This case demonstrates the feasibility and favourable outcome of tailored posterior disconnection surgery in adult DDMS, highlighting the importance of individualised presurgical evaluation and multidisciplinary management.