A systolic heart murmur was auscultated in a 2-yr-old female Sumatran orangutan (Pongo pygmaeus sumatraensis) with a slower than expected growth rate. Cardiac ultrasound revealed an 11-mm atrial septal defect. Cardiac catheterization confirmed the diagnosis. Surgical repair was performed during cardiopulmonary bypass using a pericardial patch. The bypass pump was primed with human albumin and donor orangutan whole blood of a compatible type. Hematuria occurred shortly after the initiation of cardiopulmonary bypass. Successful repair was immediately confirmed with transesophageal ultrasonography. The animal was extubated shortly after returning to spontaneous ventilation but had to be reintubated 4 hr later due to tachypnea and decreased SpO2. Additional extubation attempts failed, necessitating continuous positive pressure ventilation, monitoring, and intensive care environment. Thoracic radiographs suggested adult respiratory distress syndrome. The animal required 14 days of intensive care before extubation of the trachea was successful. After 4 wk of isolation, the orangutan was successfully reintroduced to its family group.