Spontaneous coronary artery dissection (SCAD) has been recognized as an important cause of acute coronary syndrome in women ≤ 50 years old, and up to 43% of pregnancy-associated myocardial infarction. SCAD has a strong association with extra-coronary arteriopathies, including either more common entities such as dissections, intracranial or other aneurysms, and extra-coronary and coronary arterial tortuosity or less common inherited vascular disorders such as Ehlers-Danlos syndrome, Marfan syndrome, and Loeys-Dietz syndrome, leading to the conclusion that systemic arterial disorders may underlie SCAD. Fibromuscular dysplasia is the most common extra-coronary vascular abnormality identified among these patients, also sharing a common genetic variant with SCAD. The American Heart Association, in a scientific statement regarding the management of SCAD, recommends that patients with SCAD should undergo additional evaluation with imaging techniques including either computed tomography angiography (CTA) or magnetic resonance angiography (MRA). MRA has been shown to have sufficient diagnostic accuracy in identifying extra-coronary arterial abnormalities, almost equal to CTA and conventional angiography. The aim of this review is to appraise the most recent important evidence of extra-coronary arteriopathy in the setting of SCAD and to discuss the strengths and weaknesses of various non-invasive imaging methods for screening of extra-coronary arteriopathies in patients with SCAD.