The development of new devices and treatment options has greatly increased the interest in heart valve diseases. In this context, the consideration of gender differences in diagnosis, treatment success, and prognosis is of great importance. Available data show that women and men with heart valve disease have different risk profiles, which have a significant impact on treatment outcomes and prognosis. It is the purpose of this review article to give an overview of gender-related differences in patients with valvular heart disease, regarding clinical presentation, treatment, and outcomes. In light of the emerging treatment possibilities, future research should emphasize the role of gender since both sexes benefit from tailored management.