<p>Aortic dissection (AD) represents a life-threatening cardiovascular emergency. The condition is traditionally classified according to the Stanford system: type A involves the ascending aorta, while type B is confined to the descending aorta. However, a small subset of cases defies this binary classification and is categorized as non-A non-B aortic dissection. These atypical presentations frequently involve the aortic arch or exhibit complex morphological features, such as retrograde extension or multi-territorial involvement, distinguishing them from classical type A or B dissections. The optimal management strategy for non-A non-B aortic dissection remains controversial. Current therapeutic approaches encompass open surgical repair, thoracic endovascular aortic repair (TEVAR), hybrid procedures, and conservative medical management. This review synthesizes the contemporary evidence regarding the epidemiology, pathophysiology, diagnostic challenges, and treatment modalities for non-A non-B aortic dissection, underscoring the critical importance of individualized management in therapeutic decision-making.</p>