Spontaneous coronary artery dissection is rare. In comparison with the usual myocardial infarction population, it occurs in relatively young people with a striking predilection for women. Of 83 cases that have been described, 62 were diagnosed at autopsy and 21 were found antemortem. A series of 11 patients with the antemortem diagnosis of spontaneous coronary artery dissection is reported and follow-up is provided for 16 of the 21 previously reported cases. Of the 62 autopsy cases, 10 (16%) were men (mean age 47) and 52 (84%) were women (mean age 40). Dissection of the left anterior descending coronary artery predominated in this group of patients, occurring in 80% of men and 65% of women. Of the 32 cases diagnosed antemortem, 15 (47%) were men (mean age 46) and 17 (53%) were women (mean age 38). Men were found to have dissection predominantly of the right coronary artery (73%). Women were found to have dissection predominantly of the left coronary artery system (88%). The etiology of spontaneous coronary artery dissection remains unclear. The 3 groups that can be identified are those associated with atherosclerotic coronary artery disease, those in the postpartum period and those found to be idiopathic. Of the 94 known cases, 69 (73%) occurred in women; 21 (31%) of these were associated with the postpartum state. The prognosis of patients surviving the initial event is good, with an 82% survival rate (follow-up: range 1.5 to 144 months, mean 38). The indications for medical or surgical treatment are presented. Spontaneous coronary artery dissection should be considered in the differential diagnosis of any young person sustaining an acute myocardial infarction, especially women in the postpartum state.