Background Arrhythmogenic right ventricular cardiomyopathy (ARVC) is becoming a more commonly diagnosed entity with frequent need for coincident implantable cardioverter defibrillator (ICD) therapy. Given predominant right ventricular disease with thinning of the wall, there is concern regarding the safety of transvenous lead extraction (TLE) in ARVC. Methods We performed a retrospective study of consecutive patients with ARVC undergoing TLE of ICD leads at three high-volume centers. Patient and lead characteristics, indications, outcomes, and extraction sheath (ES) use were analyzed. Results Between 1999 and 2012, more than 2,000 lead extractions were performed at the three centers. Of these, 11 patients underwent 14 extractions meeting inclusion criteria. Mean implant duration was 74.5 months (range 6-140). In 11 patients, a total of 22 leads (16 high-voltage and six pace-sense leads) were extracted in 14 procedures. The cohort was 50% male with a mean age of 45 years (range, 25-56) and mean ejection fraction 55 ± 13%. The majority (64%) of leads were extracted due to lead malfunction, three patients had an ICD lead removed for exit block, and three patients underwent TLE for infectious complications (two local, one systemic). ES assistance with laser or mechanical cutting sheaths was employed in the vast majority of cases (85.7%). All leads were removed completely. There were no major procedural complications. In five cases, lead reimplantation encountered low-amplitude R waves requiring multiple attempted lead positions before final successful implant. Conclusions This is the first reported series of TLE in ARVC patients. TLE can be performed safely and effectively in patients with ARVC by experienced operators at high-volume centers with a low complication rate.