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Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an earlyoperation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heartmay not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those ofrespiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, includingsiblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR havebeen reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery wasperformed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.