Holt-Oram syndrome is a disease with autosomal dominant inheritance characterized with cardiac defects and upper extremity skeletal anomalies. The prevalence of this syndrome is reported as 1 in 100.000 births. A 13-year-old female patient with Holt-Oram syndrome also called as Heart and Hand syndrome was presented. Tricuspid valve repairment was made in addition to the congenital cardiac defect repairment in the patient who also had tricuspid valve deficiency. The patient underwent a successful surgery.