Neuroblastoma is a common malignancy diagnosed during childhood. Metastatic neuroblastoma frequently involves regional lymph nodes, bone, and bone marrow. In contrast, neuroblastoma involvement of the inferior vena cava (IVC) is extremely rare. We report a case of neuroblastoma with IVC invasion visualized with I-123 metaiodobenzylguanidine (I-123 MIBG) scintigraphy during a diagnostic workup while a follow-up I-123 MIBG scan showed resolution of tumor uptake in the IVC with treatment.