Plasminogen activator inhibitor-1 (PAI-1) deficiency causes a rare bleeding disorder by allowing excessive fibrinolysis to occur. Knowledge of the specific type and severity of the bleeding disorder is crucial in planning a safe and appropriate treatment sequence in conjunction with a hemophilia team. This article reports the oral management of a 9-year-old female with PAI-1 inhibitor deficiency using tranexamic acid (Cyclokapron).