Spontaneous coronary artery dissection (SCAD) is a rare cause of acute coronary syndrome (ACS) that typically affects the younger and healthier female population without the typical ACS risk factors such as hypertension, diabetes, or hyperlipidemia. The clinical presentation of SCAD can be diverse and the diagnosis is typically by coronary angiography but also can require advanced imaging such as intravascular ultrasound or optical coherence tomography. Past studies have shown the atypical patient characteristics of SCAD patients among ACS patients. The main challenge is that the exact pathophysiology of SCAD is unknown. Potential pathophysiological risk factors are discussed including fibromuscular dysplasia, other arteriopathies, pregnancy and female sex hormone changes, migraines, inflammatory conditions, and stress. The current understanding of these risk factors along with potential pathophysiological mechanisms are discussed. There still remain many areas of additional investigation in understanding this rare cause of ACS.