Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by stenosis or occlusion of the terminal internal carotid arteries and the formation of abnormal collateral vessels at the base of the brain. Pediatric patients commonly present with ischemic manifestations such as seizures, transient ischemic attacks, or stroke. Early diagnosis is essential to prevent progressive neurological impairment. We report the case of a 3.5-year-old girl who presented with dysphagia and global aphasia following a prolonged generalized tonic-clonic seizure. She had a history of two previous seizure episodes over the past year. Neurological examination revealed right-sided motor weakness with hyperreflexia and an extensor plantar response. Initial neuroimaging performed after the first seizure had demonstrated focal cerebral atrophy and gliosis suggestive of an old infarct. Further evaluation with magnetic resonance imaging and magnetic resonance angiography revealed severe attenuation of the intracranial internal carotid arteries and major cerebral branches, along with the presence of basal collateral vessels, consistent with Moyamoya disease (Suzuki grade II-III). The patient was managed with antiplatelet therapy, anticonvulsants, and supportive care and was referred for digital subtraction angiography and neurosurgical evaluation for possible revascularization. This case highlights the importance of considering Moyamoya disease in young children presenting with recurrent seizures and focal neurological deficits. Early neurovascular imaging using MRI and MRA plays a crucial role in establishing the diagnosis and guiding timely referral for definitive management. Prompt recognition and appropriate intervention are essential to prevent recurrent ischemic events and improve long-term neurological outcomes.