Acute ischemic stroke in the pediatric population is rare but carries lasting and often lifelong morbidity. Thrombolysis and mechanical thrombectomy are mainstays of care in adults, yet there is very little evidence for these treatments in children. We present the case of a 4-year-old boy with complex congenital heart disease, admitted 30 min after sudden onset of an aphasia and right hemiplegia, scoring 14 on the Pediatric National Institutes of Health Stroke Scale (PedNIHSS). Non-contrast brain computed tomography (CT) showed no evidence of acute ischemia. CT angiogram demonstrated a thrombus in the M1 segment of the left middle cerebral artery. Intravenous recombinant tissue plasminogen activator (rTPA) was infused 3.5 h after onset of symptoms. An improvement was observed in the hour after rTPA, with a PedNIHSS score of 7. Digital subtraction angiography was performed approximately 9 h from the onset of symptoms, showing a complete left M1 occlusion. The patient underwent successful mechanical thrombectomy and was discharged with a PedNIHSS score of 2. This case emphasizes the importance of early recognition to direct children towards rapid diagnosis and hyperacute treatment.