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Introduction Since Prinzmetal first described a "variant" form of angina pectoris, with predominantly resting episodes of pain and cyclic severity variations, it has gradually become apparent that this clinical presentation is caused by episodes of coronary artery spasm (CAS) involving focal or diffuse changes in large and/or small coronary arteries in the presence or absence of "fixed" coronary artery stenoses. However, most clinicians have only limited understanding of this group of disorders. Areas covered We examine the clinical presentation of CAS, associated pathologies outside the coronary vasculature, impediments to making the diagnosis, provocative diagnostic tests, available and emerging treatments, and the current understanding of pathogenesis. Expert opinion CAS remains a poorly understood, often debilitating and substantially under-diagnosed condition, which occurs more frequently in women than in men, and is poorly correlated with all conventional coronary risk factors except smoking. Many patients presenting with CAS crises have non-diagnostic ECGs and normal serum troponin concentrations, but CAS can be suspected on the basis of history and association with migraine, Raynaud's phenomenon and Kounis syndrome. Definitive diagnosis requires provocative testing at coronary angiography. Treatment still centres around the use of calcium antagonists, but with greater understanding of pathogenesis, new management options are emerging.