Introduction: -The purpose of this case presentation is to convey the importance of anesthetic plan in pediatric Kawasaki disease patients who present with severe left ventricular dysfunction. Case: A 2 year old male presented with an episode of respiratory failure of unknown etiology. The patient was transferred from an outside facility to our institute for respiratory failure of unknown etiology with cardiomegaly in chest x- ray. Management and outcome: Following initial stabilization the patient remained dyspneic. In the emergency department a bedside chest x-ray demonstrated cardiomegaly. A formal cardiac transthoracic echocardiography was obtained which demonstrated findings of coronary vessel aneurysm and hypokinetic myocardium. The patient was transferred to pediatric cardiothoracic surgery after confirming left anterior descending artery(LAD) occlusion with retrograde filling form right coronary artery. His abnormality was ultimately repaired by arterial grafting. Discussion: The majority of congenital heart diseases are diagnosed near the time of birth but may present suddenly in otherwise well pediatric patients. Clinical suspicion for heart disease should be in the differential diagnosis of an unresponsive pediatric patient without cardiac murmur and respiratory failure. We review the presentation and clinical findings in patients with Kawasaki disease with severe left ventricular dysfunction.