A 37-year-old primigestational female with prenatal diagnosis of tetralogy of Fallot (ToF) with pulmonary valve agenesis at 26 weeks of gestation. Genetic ultrasound screening with low risk for chromosomal abnormalities was performed. FISH and 22q11 deletion were negative. Fetal echocardiogram was performed with findings of an overriding aorta under a wide subaortic ventricular septal defect, which was more evident with colour Doppler assessment. No pulmonary valve was observed (figure 1A). There was a severe dilatation of the pulmonary artery trunk with retrograde flow and enlargement of the right atrium (figure 1B). Cardiac axis was altered. The findings were suggestive of ToF with pulmonary valve agenesis. No other malformations in other structures were identified. A Caesarean section was performed and a female infant weighting 6.5 pounds was delivered. The diagnosis was confirmed postnatally with a thoracic echocardiogram. Transfontanelar, biliar and liver ultrasound were normal and showed no evidence of other pathological malformations. TOF is the most common cyanotic congenital heart disease in newborns. 1. Classic features include a perimembranous ventricular septal defect, a superimposed aorta, right ventricular hypertrophy, and pulmonary stenosis (1,2). TOF with agenesis of the pulmonary valve is a very rare variant that affects approximately 3% of patients with TOF. 2. Diagnosis is made prenatally by ultrasound (2). An accurate prenatal diagnosis is essential to be able to plan postnatal management, since these patients require specialised care at birth. We present a case of a patient with TOF with pulmonary valve agenesis that was diagnosed prenatally in Bogotá, as the first reported case in Colombia. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.