One case of a 62-year-old female patient with right ventricular malformation complicated by sick sinus syndrome was reported. Due to the high risk of pacemaker infection associated with long-term glucocorticoid use, an active-fixation leadless pacemaker was implanted via the internal jugular vein approach under general anesthesia with transesophageal echocardiography guidance. The procedure was successfully completed. Postoperatively, the patient's recurrent palpitations and dizziness resolved completely. Electrocardiography revealed a pacing rate of 60 bpm with normal pacing and sensing functions. This case indicates that implantation of an active-fixation leadless pacemaker via the internal jugular vein approach is safe and feasible for patients with right ventricular malformation, complicated by sick sinus syndrome. Preoperative multimodal imaging assessment and meticulous intraoperative manipulation are the key factors for successful outcomes.