Background: Patients with injection drug use (IDU) have increased risk of developing infective endocarditis (IE), however data of these patients are scarce and need to be explored further. This study aims to describe the clinical characteristics and outcomes among patients with IDU-IE.Methods: Data of adults with IDU-IE and non-IDU-IE, treated between 2008-2017 at the Karolinska University Hospital in Stockholm were obtained from the Swedish National Registry of Infective Endocarditis. Clinical characteristics, microbiological results, treatment durations, results from echocardiography and in-hospital mortality were compared between the groups. Results: In total, 165 (32%) of the included patients had IDU-IE. Patients with IDU-IE were younger than the patients with non-IDU-IE (mean age IDU-IE: 41.6 years; non-IDU-IE: 64.3 years; P<0.01). No difference in distribution of genders was observed, 33% women in both groups. History of previous IE (n=49, 30%) and vascular phenomena (n=101, 61%), were more common among patients with IDU-IE while prosthetic heart valves (n=83, 23%) and known valvular disease (n=78, 22%) were more common among patients with non-IDU-IE (P<0.05). Aetiology of Staphylococcus aureus (n=121, 73%) and tricuspid (n=91, 55%) or pulmonary valve (n=7, 4%) vegetations were more common among IDU-IE (P<0.05). The overall incidence of IDU-IE decreased during the study period, while the incidence of definite IE increased (P<0.01). Conclusions: Patients with IDU-IE were younger, frequently presenting with right-sided vegetations and S. aureus aetiology, and were less frequently treated with surgery. Patients with IDU-IE had higher prevalence of vascular phenomena and history of previous IE, aspects that are important for improved management of this population.