Congenital left ventricular diverticulum is a rare cardiac abnormality consisting of a localized outpouching from the free wall of the cardiac chamber. It is usually asymptomatic. However, complications such as infective endocarditis, arrhythmias, and embolism may occur. Medical ultrasound, echocardiography, CT angiography, MRI and invasive ventriculography are diagnostic tools used to identify congenital left ventricular diverticulum. Surgical resection is the treatment of choice in symptomatic patients, whereas the management of asymptomatic patients often represents a therapeutic dilemma. We report the case of a symptomatic left ventricular diverticulum on a 3-day-old female with Cantrell’s syndrome treated surgically. Postoperative course was uneventful. The patient was discharged with a good clinical condition. The prognosis of this malformation is poor if not diagnosed in the perinatal period. A diagnosis can be suspected with echocardiography; however, a CT scan allows a complete study of the problem. The treatment is always surgical with a good postoperative prognosis.