Extended-spectrum β-lactamases (ESBLs) have been increasingly described worldwide, especially among Enterobacteriaceae isolates, and recently not only in the nosocomial, but also in the community setting. Bloodstream infections (BSIs) caused by ESBL-producing Enterobacteriaceae have been associated with increased rates of treatment failure, mortality and hospital costs. Any delay in the initiation of adequate antibiotic therapy is potentially lethal for patients with BSIs caused by ESBL-producing Enterobacteriaceae. The awareness of changes in bacterial resistance patterns, the careful knowledge of risk factors for ESBL infection and of factors facilitating adverse outcome, giving attention to local epidemiology, can improve the efficacy of empirical treatment protocols. The aim of this review is to focus on the main characteristics of BSIs caused by ESBL-producing Enterobacteriaceae, with particular emphasis on risk factors for these infections and factors related to mortality.