A 6 months old male infant came with lethargy and poor feeding. On evaluation there was bradycardia. Positive family history of sibling death and hearing loss in another sibling were present. ECG shows long QT interval and B/L sensori-neural hearing loss. On the basis of long QT and hearing defect a diagnosis of Jervell- Lang - Nielsen syndrome was suspected and child was started on beta blocker therapy. epicardial pacemaker insertion was done and child was given discharge.