Massive hemoptysis is a common complication in patients with cystic fibrosis (CF) and is associated with significant morbidity and mortality. Conventional treatment with antibiotic therapy and early bronchial artery embolization (BAE) is usually successful in achieving hemostasis in the majority of patients. Recombinant activated factor VII (rFVIIa), originally developed for use in patients with hemophilia, has emerged as a general hemostatic agent that is potentially useful in the management of many life-threatening bleeding conditions. In this article, we present four patients with CF lung disease and massive hemoptysis who were treated successfully with rFVIIa. We suggest that in patients with CF who present with massive hemoptysis, the use of rFVIIa can be considered in patients with refractory hemoptysis despite conventional therapy or as a temporizing therapy when BAE is not immediately available.