BACKGROUND Upper extremity deep vein thrombosis (UEDVT) constitutes around 10% of all DVT, and can cause both pulmonary embolism (PE) and postthrombotic syndrome (PTS) in the arm. The incidence of secondary UEDVT is increasing due to widespread use of central venous catheters in patients with cancer and other chronic diseases. The safety and efficacy of using new direct acting oral anti coagulants (DOAC) in the treatment of UEDVT has not been systematically evaluated. Our aims were to evaulate efficacy, safety, and risk of recurrence of venous thromboembolism (VTE) during DOAC treatment in UEDVT patients. METHODS Data from the Swedish national anticoagulation registry (AuriculA) was retrospectively evaluated for all 55 patients (27 men aged 23-86 years, and 28 women aged 18-75 years) treated with DOAC because of UEDVT between 2012 and 2015 in the southernmost hospital region of Sweden with 1.3 million inhabitants in 2016. Patients were followed for 6 months. RESULTS During 6 months after institution of DOAC treatment there was one recurrence (2%) of DVT during treatment and two (4%) recurrences after cessation of treatment. No patient died, whereas one (2%) suffered a clinically relevant nonmajor bleeding. CONCLUSIONS DOAC can be used in the treatment of UEDVT patients with acceptable efficacy and safety.