Congenital left atrial aneurysm is a very rare congenital cardiac anomaly, and very few cases have been reported with neonatal presentation. Despite the congenital origin of this condition, it may remain asymptomatic and patients may not present until their third decade of life. Even in asymptomatic cases, prompt surgical intervention is indicated for the prevention of fatal thromboembolic events such as stroke. In neonates and infants presenting early in life, surgical intervention is urgent to relieve respiratory distress symptoms. Herein, we introduce the case of a 3-week-old neonate who presented with severe respiratory distress symptoms requiring prompt intubation and mechanical ventilation. Diagnosis of congenital left atrial aneurysm was established and confirmed by transthoracic echocardiography and computed tomography angiography. The patient underwent surgical resection of the aneurysm using cardiopulmonary bypass and cardiac arrest. The patient was discharged from the hospital after three weeks with normal echocardiography.