Congenital heart disease is a lifelong disease that results from a heart defect or structural anomaly. Signs and symptoms of heart disease vary by age and developmental stage of the pediatric patient. Thorough history and physical examination guide the nurse practitioner to determine the need for further evaluation, referral, and collaboration with acute care and cardiac specialists to manage this complex chronic illness. Using an illustrative case, this article highlights the signs and symptoms concerning for congenital heart disease; suggests available resources; and encourages frequent communication and collaboration to improve outcomes of this diverse, vulnerable population throughout the life span.