INTRODUCTION: Many aspects of hemostasis, both primary and secondary, as well as fibrinolysis display sex differences. From a clinical viewpoint, certain differential phenotypic presentations clearly arise within various disorders of thrombosis and hemostasis. Areas covered: The present mini-review summarizes selected clinical entities where sex differences are reflected in both frequency and clinical presentation of hemostasis disorders. Sex differences are discussed within the settings of cardiovascular disease, including coronary artery disease and ischemic stroke, venous thromboembolism and inherited bleeding disorders. Moreover, pregnancy and labor present particular challenges in terms of increased thromboembolic and bleeding risk, and this is also summarized. Expert commentary: Available knowledge on sex differences in risk factors and clinical presentation of disorders within thrombosis and hemostasis is increasing. However, more evidence is needed to further clarify different risk factors and treatment effect in men and women, both as regards to cardiovascular disease and venous thromboembolism. This should facilitate improved gender guided risk stratification, and prevention and treatment of these diseases. Finally, risk assessment during pregnancy remains a challenge; this applies both to thromboembolic risk assessment during normal pregnancy and special care of women with inherited bleeding disorders during labor.