We studied the long-term outcome after BAE for life-threatening hemoptysis in patients with CF. Data from pulmonary function tests were available for 18 of the 25 patients followed. A case-control comparison revealed that these 18 patients died sooner than hemoptysis-free patients with CF matched for age, sex, and pulmonary function (p less than 0.02), with the excess mortality occurring within the first three months after BAE. Of all 25 patients followed, six died of cardiorespiratory failure within three months of BAE; in two of them, hemoptysis was a contributing cause of death. The 19 patients who lived more than three months after BAE had a mean survival after embolization of 3.5 years (five were still alive at the end of the study). Most patients experienced long intervals (greater than 1 year) free of major hemoptysis. Extended follow-up (mean, 35 months) revealed a higher incidence of recurrent severe bleeding than previously reported for 13 of these patients followed a mean of 11 months. Repeat BAE for severe recurrence was performed successfully in eight of nine patients, without complication.