Complete atrioventricular septal defect (CAVSD) is known to be associated with Down syndrome (DS) and to be a major cause of infantile death in these patients. Recently several leading articles reported that complete surgical repair before 6 months of age is a relatively low-risk procedure leading to a dramatic improvement in outcome. Most surgical reviews, however, fail to describe their selection criteria and to include patients who die early in infancy or who are unfit for surgery for some or other reason. A retrospective descriptive study was done of all children with CAVSD treated over a period of 15 years from 1980 to 1994 at our institution. The aims were to describe the profile of children with CAVSD in our population, and to evaluate screening procedures and treatment received versus outcome. Thirty-three patients were included in the study, 25 with DS and 8 without. The basic skills of careful auscultation, interpretation of chest radiograph and electrocardiograms (ECGs) are all 100% sensitive in alerting the physician to this condition. Careful screening, especially of DS babies, is essential to ensure early optimal intervention. Echocardiography is the least invasive, most informative investigation to establish a definite diagnosis. The Rastelli anatomical classification is highly indicative of outcome. Children in the Rastelli C group showed a universally dismal prognosis. Digitalis and furosemide are still the mainstay of medical treatment. No additional benefit was observed with the limited introduction of angiotensin-converting enzyme (ACE) inhibitors. Complete repairs were undertaken earlier and more frequently in recent years, but limited resources had led to the majority of patients not receiving surgery at all. There was insufficient urgency to aim for early complete repair, and more than 20% of children died before even being considered for surgery. This study is the first in South Africa to describe our own patient population and to evaluate local practice. Adequate screening of babies (especially those with DS), with early referral for complete repair in the first 6 months of life, remains the goal of optimal management.