Tricuspid atresia (TA) is a rare congenital heart defect in which the right atrioventricular connection, the tricuspid valve, is absent. As a result, there is no direct communication between the right atrium and right ventricle. Surgical treatment, including the Fontan procedure, is indicated yet palliative, leaving patients with various lifelong complications. A comprehensive literature review revealed a paucity of evidence-based education on the identification, evaluation, management, treatment, and life span implications of TA. We present a case of TA from birth through adulthood, while simultaneously assessing the literature, to report the most current evidence relative to living with TA after surgical palliation. In addition, the embryology, methods of prenatal and postnatal diagnosis, potential complications, management, anticipatory guidance, and educational needs of both parents and patient are discussed.