A spectrum of distinctive clinical presentations and electrocardiographic patterns have been recognized in neonates with ventricular arrhythmias. These may range from an incidental finding on a routine physical to cardiovascular collapse due to ventricular fibrillation. It has become increasingly important that the clinician considers ventricular tachycardia in the neonate with tachycardia when the QRS normal does not appear normal. In general, isolated premature ventricular depolarizations, couplets and non-sustained ventricular tachycardia in the absence of heart disease are associated with a favorable prognosis. Most of these arrhythmias tend to resolve during the first month of life. Conversely, sustained ventricular arrhythmias associated with ischemia, myocarditis or ventricular tumors are associated with a guarded prognosis. Treatment is based on the definition of associated cardiovascular disease, support of hemodynamic status and the judicious use of antiarrhythmic agents. Finally, there has been an increased recognition of idiopathic forms of ventricular tachycardia in the neonate which are associated with a favorable prognosis and may not require pharmacologic treatment. This review will discuss these arrhythmias in neonates, associated forms of cardiovascular disease, current treatment options and long-term prognosis.