Diseases of the supra-aortic trunks (SATs) encompass a broad and heterogeneous group of arterial disorders, ranging from classic inflammatory vasculitis to recently recognized, non-atherosclerotic entities. While atherosclerosis remains the predominant cause of SATs stenosis, up to 10% of carotid or subclavian artery lesions occur in patients without established cardiovascular risk factors. These rare conditions are increasingly identified through advances in vascular imaging, which now allow detailed analysis of the vessel wall. Among the most notable emerging entities are Transient Perivascular Inflammation of the Carotid artery (TIPIC) syndrome, carotid web, and Eagle syndrome. Their recognition has reshaped our understanding of carotid and subclavian artery pathology, revealing a spectrum that extends beyond inflammatory and atheromatous mechanisms to include structural, mechanical, and developmental abnormalities. This comprehensive overview explores rare and emerging arterial diseases involving the SATs, highlighting recent diagnostic and therapeutic insights. We emphasize the pivotal role of multimodal imaging in differentiating these entities and propose an integrated diagnostic approach combining clinical, imaging, and pathophysiological perspectives.