Carotid artery dissection is a cause of stroke, especially in young and middle-aged patients. A dissection occurs when there is an intimal tear or rupture of the vasa vasorum, leading to an intramural hematoma, which is thought to result from trauma or can occur spontaneously, and is likely multifactorial, involving environmental and intrinsic factors. The clinical diagnosis of carotid artery dissection can be challenging, with common presentations including pain, partial Horner syndrome, cranial nerve palsies, or cerebral ischemia. With the use of noninvasive imaging, including magnetic resonance and computed tomography angiography, the diagnosis of carotid dissection has increased in frequency. Treatment options include thrombolysis, antiplatelet or anticoagulation therapy, endovascular or surgical interventions. The choice of appropriate therapy remains controversial as most carotid dissections heal on their own and there are no randomized trials to compare treatment options.