Numerous studies have described the pathophysiology, clinical manifestations, and treatment of the many forms of congenital heart disease (CHD), but none has specifically addressed the reasons CHD patients present to the Emergency Department (ED). The objective of this study was to provide a descriptive analysis of the ED presentations of acute and seriously ill pediatric CHD patients. We intended to capture a subset of acutely ill CHD patients who had presenting signs and symptoms that were potentially attributable to their underlying CHD. Recognizing the more common presentation patterns for patients with CHD may be of benefit. Adhering to the basic principles of airway, breathing and circulatory (ABC) management is essential when caring for critically ill CHD patients. Patients with complex CHD are often very difficult to correctly diagnose and manage in the ED. They often require extensive inpatient observation and evaluation. A low threshold for inpatient management of these high-risk patients is warranted.