Background: Anomalous aortic origin of coronary artery is a rare finding, with varied presentation and symptomatology. Increasingly recognized by cardiac imaging, when found it raises questions about the appropriate approach and management.Case presentation: We present a case of an 11-year-old female who presented with episodes of shortness of breath, angina, and syncope during exercise. Further investigation demonstrated episodes of nonsustained ventricular tachycardia on Holter and coronary angiotomography revealed that the left coronary artery had an anomalous origin from the right cusp with initial short intramural segment and significant external compression in its initial course between the aorta and the pulmonary artery. The patient was submitted to surgical correction with dissection

Kawasaki disease (KD) is an inflammatory condition that may affect genetically predisposed individuals in pediatric ages after infectious/environmental triggering. An infrequent finding associated with KD is ascending aortic aneurysm during or after the acute phase of the disease. In this Multimedia presentation, we describe a three-year-old girl submitted to surgical treatment.