Acute ischemic stroke in patients younger than the age of 50 years is a rare occurrence that results in high mortality and substantial loss of functional years of life. Internal carotid artery dissection (CAD) presents a rare, but serious condition that needs to be fully evaluated and carefully treated, as it may lead to an acute ischemic stroke in all, but mostly in younger patients. A possible cause for CAD, the carotid artery type of Eagle syndrome (ESy), is atypical and underrecognized. In this case report we present a case of a young patient with carotid artery type of ESy, resulting in a severe acute ischemic stroke. Only recognition of such a syndrome in its early symptomatic phase could allow appropriate management to prevent this kind of a deleterious outcome.