Tetralogy of Fallot (TOF) is one of the most common cyanotic congenital cardiac malformations and is characterized by four cardinal features: a large VSD; often a dynamic right ventricular outflow tract obstruction (RVOTO); an overriding aorta; and right ventricular hypertrophy (RVH). The severity of the RVOTO, the pressure gradient between the ventricles, and the proportion of the aorta overriding the VSD determine the clinical presentation and the severity of this condition. There are several variants of this condition, that range from mild to very severe, which is distinguished by the degree of the cyanosis. Exaggeration of the condition might occur, which is known as hypercyanotic spells, or tet spells. These spells result in worsening of the right-to-left shunt that leads to a worsening of cyanosis, and if left untreated it will result in death. In the current era, more and more cases are diagnosed antenatally due to better screenings during the pregnancy but also due to improved diagnostic procedures. The majority of cases are managed by a single-step surgical correction. Due to an improved surgical technique, most cases of TOF could be completely repaired within the first 6 months of life. Although, if the case is severe, a two-step procedure must be done, first-step would be an insertion of a palliative shunt, and after few months the second-step which is a complete correction.