Bleeding into the corpus luteum following ovulation rarely has clinical significance in healthy women, but may lead to life-threatening hemorrhage in women with congenital or acquired bleeding disorders. Women who are at an increased risk for corpus luteum hemorrhage (CLH) can be divided in two categories; first, those taking anticoagulants because of a thrombotic disorder; and second, women with congenital bleeding disorders. The management and prevention of CLH is still unsettled and the literature dealing with this problem is based on case reports only. This review focuses on the pathophysiology, clinical presentation, diagnosis and treatment options of an acute bleeding event and prevention modalities of CLH in women with bleeding disorders.