Although the incidence of sudden cardiac death (SCD) is greater in men than in women, it represents an important mode of death also in the female gender. Sex-related differences have been identified not only in the prevalence of the phenomenon, but also in risk factors and etiology of SCD. The peripartum period represents a peculiar trigger of SCD in women with underlying cardiovascular substrates. Several lesions, both congenital and acquired, may be identified at postmortem in SCD victims, including coronary artery, myocardial, valve, and aortic diseases. While the incidence of SCD increases progressively with age in adult elderly women to reach a 1:1 male:female ratio after the age of 80 years, mostly due to the increasing incidence of atherosclerotic disease in the postmenopausal period, SCD in young women is a rare event and usually associated with non-atherosclerotic disease, such as mitral valve prolapse, spontaneous coronary dissection, myocarditis, inherited cardiomyopathies and congenital heart diseases. The heart can be found structurally normal and inherited ion channel diseases are often implicated. Gender differences in the risk of SCD deserve further attention, since they affect the evaluation of interventions designed to reduce the rate of female SCD.