Despite being one of the most common congenital cardiac abnormalities, the treatment of critical left ventricular outflow tract obstruction (LVOTO) in the neonate and infant remains a significant challenge. Critical LVOTO in the neonate and infant includes continuous and diverse spectrum of anatomic diagnoses ranging from hypoplastic left heart complex to isolated aortic valvular stenosis with otherwise normally formed left heart structures. Depending on related anatomic features, there are multiple single ventricle and biventricular management strategies available for patients with critical LVOTO; all of which carry significant risk of death and requirement for future interventions. Because of the wide array of morphologic lesions and physiologic manifestations, application of the currently available published data to guide decision making in the individual patients can be challenging. Therefore, the aim of this review is to describe the different therapeutic strategies that can be applied to neonates presenting with critical LVOTO taking into consideration each patient's unique physiologic and anatomic findings.