A case is presented of a young girl with symptoms of the Holt Oram syndrome which is a combination of a trial sPetal defect, characteristic congenital anamolies of the thumbs, wrists and forearms and alteration in the E.C.G..The syndrome occurs in both sexes equally and affects all races. It is inherited as autosomal dominant character. The causes can be embryologically explained, but whether it is purely genetic or whether there are precipitating factors has not yet been determined. The most common combination of defects is ASD of secundum type and anomalies of the thumb. Members of the same family have similar cardiac and bone lesions and these became more severe trom one generation to another. If chromosomal aberrations are found, the anomalies will be more severe.