Infants of birth weight ≤2500 g are termed low birth weight (LBW). These children often have considerable morbidity from prematurity and intra-uterine growth restriction. Additionally, LBW infants have increased risk for cardiac and noncardiac congenital anomalies and may require surgery. Primary rather than palliative surgical repair of cardiac lesions has been preferred in recent years. However, LBW remains a risk factor for increased mortality and morbidity after open-heart surgery (OHS). There is a paucity of information about the anesthetic challenges presented by LBW infants undergoing OHS. This review summarizes the perioperative issues of relevance to anesthesiologists who manage these high-risk patients. Emphasis is placed on management concerns that are unique to LBW infants. Retrospective data from the authors' institution are provided for those aspects of anesthetic care that lack published studies. Successful outcome often requires substantial hospital resources and collaborative multi-disciplinary effort.