Three patients from different centers with pacemaker or ICD leads endocardially implanted in the left ventricle are described. All leads, two ventricular pacing leads and one ICD lead, were inserted through a patent foramen ovale or an atrial septum defect. The diagnosis was made 9 months, 14 months, and 16 years, respectively, after implantation. All patients had right bundle branch block configuration during ventricular pacing. Chest X ray was suggestive of a left-sided positioned lead except in the ICD patient. Diagnosis was confirmed with echocardiography in all patients. One patient with a ventricular pacing lead presented with a transient ischemic attack at 1-month postimplantation. During surgical repair of the atrial septum defect 14 months later, the lead was extracted and thrombus was attached to the lead despite therapy with aspirin. The other patients were asymptomatic without anticoagulation (9 months and 16 years after implant). No thrombus was present on the ICD lead at the time of the cardiac transplantation in one patient. We reviewed 27 patients with permanent leads described in the literature. Ten patients experienced thromboembolic complications, including three of ten patients on antiplatelet therapy. The lead was removed in six patients, anticoagulation with warfarin was effective for secondary prevention in the four remaining patients. In the asymptomatic patients, the lead was removed in five patients. In the remaining patients, 1 patient was on warfarin, 2 were on antiplatelet therapy, and in 3 patients the medication was unknown. After malposition was diagnosed, three additional patients were treated with warfarin. In conclusion, if timely removal of a malpositioned lead in the left ventricle is not preformed, lifelong anticoagulation with warfarin can be recommended as the first choice therapy and lead extraction reserved in case of failure or during concomitant surgery.