We report a patient with a congenital mediastinal dumbbell neuroblastoma (NB) presenting as respiratory distress at birth rather than a neurologic deficit. The tumor did not respond to low-dose chemotherapy, but radiotherapy and surgery were effective. The patient was observed postoperatively without further therapeutic intervention considering the favorable biologic factors despite the appearance of liver metastases, which ultimately regressed spontaneously. The authors believe that most congenital NBs have favorable outcomes with a possibility of spontaneous regression, and that an understanding of the biologic features of tumors is important to avoid overtreatment of lesions with a favorable prognosis.