Aortic dissection is a rare, potentially catastrophic vascular emergency. Early recognition of the clinical manifestations, rapid confirmation using imaging modalities, urgent administration of appropriate medication and expedient selection of definitive long-term therapy are key to preserving life and reducing morbidity. In recent years it has become increasingly clear that there is a relation between cocaine and aortic dissection. Cocaine serves as both a predisposing factor to aortic dissection due to its effect on aortic connective tissue and as a precipitating factor due to its propensity to produce abrupt and severe hypertension. While similarities exist in the clinical features and diagnostic methods between cocaine-related aortic dissection and aortic dissection unrelated to cocaine use, there are important differences in management between these two syndromes which are rooted in the pharmacology and physiology of cocaine. An understanding of these differences is key to effective early and long-term management of cocaine-related aortic dissection.