Holt-Oram syndrome also known as heart and hand syndrome, first reported in 1960. It is a rare, inherited, an autosomal dominant disorder with mutation in TBX5. As the name suggests, the feature involves skeletal abnormality mainly involving upper limb, that is, upper-extremity malformations involving radial, thenar, or carpal bones; congenital heart diseases like, atrial-septal defect and ventricular septal defect and conduction problems. This syndrome may also involve other part of skeletal structure. We hereby report a case of a patient who was suffering from this syndrome scheduled for right radial head excision.