Budd-Chiari syndrome due to the tip of an internal jugular tunneled dialysis catheter malposition in inferior vena cava or hepatic vein is a rare complication. We aimed to present our experience and compare it with the previous reports to highlight the clinical features and the optimal management. A 57-year-old female with history of ANCAp vasculitis, treated by hemodialysis in the last 2 years on a right internal jugular vein tunneled catheter was admitted for pain in the right upper quadrant. A subacute Budd-Chiari syndrome due to catheter malposition was diagnosed. The catheter was removed, and a new tunneled hemodialysis line was inserted in the right internal jugular vein with the tip at the junction of right atrium with superior vena cava. Anticoagulation with apixaban 2.5 mg twice daily was started after catheter replacement and the patient was discharged. At 1 month follow-up the patient had no symptoms, and the ultrasound revealed the absence of the thrombus in the inferior vena cava. Imagining monitoring for malposition after insertion or in a clinical context suggestive for Budd-Chiari syndrome is essential for early diagnosis and treatment. In our case, anticoagulation with apixaban and prompt catheter replacement resulted in Budd-Chiari syndrome resolution.