Two women, 34 and 54 years old, died suddenly from acute coronary occlusion due to spontaneous dissecting haematoma (aneurysm) involving the entire left coronary artery and the right coronary artery, respectively. In the older woman, cystic medial necrosis of the coronary tunica media was observed. The younger case was the only sudden death due to dissecting hematoma among 120 cases in the Registry of "juvenile sudden death" in the Veneto Region, Italy. From a review of the literature, following the first description in 1931, we collected 97 cases with the following characteristics: mean age in women, 39 years, in men, 48 years; only significant risk factor: peripartum (34% of women); coronary arteries involved: isolated left anterior descending (52.5%, equally distributed among women and men), right (24%, mainly men), left (13.5%, only women), multiple vessels (8%), left circumflex artery (2%). The clinical presentation was cardiac arrest with sudden death in 49.5%, acute, non-fatal myocardial infarct in 33%, and acute fatal myocardial infarct in 17.5% of the cases. The prognosis is very poor, and emergency medical or surgical myocardial revascularization is rarely feasible.