We report a patient with a congenital mediastinal dumbbell neuroblastoma (N
B) presenting as respiratory distress at birth rather than a neurologic def
icit. The tumor did not respond to low-dose chemotherapy, but radiotherapy
and surgery were effective. The patient was observed postoperatively withou
t further therapeutic intervention considering the favorable biologic facto
rs despite the appearance of liver metastases, which ultimately regressed s
pontaneously. The authors believe that most congenital NBs have favorable o
utcomes with a possibility of spontaneous regression, and that an understan
ding of the biologic features of tumors is important to avoid overtreatment
of lesions with a favorable prognosis.