Aneurysms of the extracranial internal carotid artery (ICA) in pediatric patients are rare and mostly treated surgically. The authors present the case of a child with a giant extracranial ICA aneurysm presenting with gradual airway compression. A plan was made to explore the aneurysm surgically and straighten the associated carotid artery kinks during the operation to enable further endovascular treatment if primary reconstruction of the carotid artery proved not to be possible. Finally, the aneurysm was successfully treated with staged hybrid intervention, which consisted of surgical correction of parent artery kinks followed by endovascular implantation of stent grafts. The child tolerated the procedure well without adverse events. A craniocervical CT angiogram at 5 months showed patency of the stent construct and relief of airway compression. However, a follow-up CT angiogram at 14 months showed total occlusion of the ipsilateral carotid artery without clinical sequelae and complete cessation of airway compression. The management of this was unique since the patient is the first reported case of clopidogrel hyporesponsiveness in a child undergoing neurovascular intervention with prasugrel monotherapy. Point-of-care testing-based adjustment of prasugrel necessitated education of the patient's family regarding the loss of effect of the prasugrel tablets upon crushing and contact with air.