Transient cerebral arteriopathy is a frequent cause of childhood arterial ischemic stroke. Differentiating this condition from intracranial carotid artery dissection is challenging but important for initial treatment. We describe 4 cases from the International Pediatric Stroke Study of intracranial carotid artery dissection, initially misdiagnosed as transient cerebral arteriopathy. Presentations were abrupt, with focal neurological deficits in 4, preceding headache in 3, and minor trauma in 1. Infarcts involved the anterior circulation, and magnetic resonance angiography showed unilateral arterial stenosis/occlusion. None had evidence of dissection. All received anticoagulation or thrombolysis. Three died from refractory intracranial hypertension. Intracranial carotid artery dissection was confirmed postmortem (n = 3) and on dedicated MR wall imaging showing intramural hematoma (n = 1). In differentiating transient cerebral arteriopathy from intracranial carotid artery dissection, routine magnetic resonance angiography is unreliable and adjunctive conventional angiography, gadolinium magnetic resonance angiography, or dedicated MRI wall imaging should be considered.