A 62-year-old female was admitted with a chief complaint of transient syncope on exertion. Angiography in right ventricle revealed a defect caused by an anomalous muscle bundle and a pressure gradient of 151 mmHg was observed between the two chambers by cardiac catheterization. Resection of the anomalouse muscle bundle was undertaken using a lower median sternotomy starting at the 2nd intercostal space level and through the outflow tract right ventriculotomy. Patch plasty was also undertaken in the outflow tract. Post-operative course was uneventful and pressure gradient had disappeared at the post-operative catheterization. A rare case of DCRV in a 62-year-old patient with a pressure gradient of 151 mmHg in the right ventricle was reported.