- Objectives: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart defect. Children with this anomaly are usually asymptomatic at birth and develop symptoms later on in life, which may mimic myocarditis. We sought to delineate clinical, laboratory, and epidemiological aspects of this anomaly. Methods: A retrospective analysis of children with ALCAPA evaluated in a tertiary medical center in southern Israel was performed. A computerized search for all patients with the diagnosis of ALCAPA between 2000 and 2011 was performed. The medical records were reviewed; demographic, clinical, and laboratory data were extracted. Results: A total of 9 patients were included. In 4 patients, acute deterioration required evaluation in the pediatric emergency medicine department; in all 4, the initial clinical suspicion was myocarditis. Failure to thrive was recorded in 7 (77.7%) of the 9 patients and asthma or wheezing were recorded in 5 (55.5%) of the 9 patients. Normal heart size was recorded in 4 (44.4%) of the 9 patients. Electrocardiographic abnormalities were present in all of the patients (100%). A total of 141,675 births were recorded during the study period, giving an incidence of at least 1 case (0.00635%) per 15,741 births. Conclusions: Children evaluated in the emergency medicine department with suspected myocarditis should be evaluated specifically for ALCAPA. The clinical findings that should raise the suspicion of this anomaly are failure to thrive and either a diagnosis of asthma or recurrent wheezing.