RATIONALE: Congenital anomalies of the aortic arch encompass a range of malformations that may remain clinically silent or present with severe respiratory or esophageal symptoms. These anomalies are crucial to recognize due to their association with vascular rings, congenital heart disease, and chromosomal abnormalities, which have significant implications for prognosis and management. This case report highlights a rare instance of tetralogy of Fallot (TOF) with an aortic arch anomaly, emphasizing the importance of accurate imaging and surgical planning. PATIENT CONCERNS: A 4-year-old male child presented with easy fatigability, dyspnea on exertion, and refusal to feed. Clinical evaluation revealed a grade 2 end-systolic murmur, cardiomegaly, and right ventricular enlargement. DIAGNOSES: Diagnostic imaging confirmed TOF with bilateral anomalous origin of the vertebral arteries from the aortic arch. INTERVENTIONS: Surgical repair was successfully performed, involving Sauvage patch closure of the ventricular septal defect and reconstruction of the right ventricular outflow tract (RVOT) with a pericardial patch. OUTCOMES: This case underscores the diagnostic and surgical challenges posed by aortic arch anomalies in TOF patients. LESSONS: Early recognition and precise imaging are critical for effective management and improved outcomes. The study also highlights the importance of understanding vertebral artery variations for both diagnostic and surgical interventions.