BACKGROUND To identify the clinical characteristics and risk factors for mortality of patients with cefepime-resistant Pseudomonas aeruginosa (FRPa) bacteremia. METHODS This retrospective study analyzed adult patients with FRPa bacteremia hospitalized between January 2006 and December 2011. RESULTS Seventy eight patients (46 male, 32 female; mean age: 72.2 ± 14.1 years) were included. Of them, 46 (59.0%) had ventilator use and 45 (57.7%) had intensive care unit stay. All the bacteremia episodes were health-care associated or hospital acquired, and 55.1% of FRPa blood isolates were multidrug resistant. The sources of bacteremia were identified in 42 patients (53.8%), with pneumonia being the most common one (28/42; 66.7%). The mean interval between admission and the sample date of the first FRPa-positive blood culture was 45.8 ± 52.6 days. The mean Pittsburgh bacteremia score was 5.0 ± 3.4. The 15-day and 30-day mortality rates were 50.0% and 65.4%, respectively. Patients (41; 52.6%) on appropriate antibiotic therapy within 72 hours of the first FRPa-positive blood culture had a higher 30-day survival rate than those without (48.8% vs. 18.9%, p = 0.011 by log-rank test). Multivariate analyses revealed that a higher Pittsburgh bacteremia score was an independent risk factor for either 15-day (p = 0.002) or 30-day mortality (p = 0.010), and appropriate antibiotic therapy within 72 hours was an independent protecting factor for either 15-day (p = 0.049) or 30-day mortality (p = 0.017). CONCLUSIONS FRPa bacteremia had a high mortality rate. The disease severity and appropriate antimicrobial therapy within 72 hours of positive blood culture were related to the patients' outcome.