Neuroblastoma is the most common extracranial solid tumor in children. Approximately half of the patients with Stage MS disease have tumor regression, but 10-15% will have progression of disease. BRAF mutations are common in some cancers, such as melanoma and pediatric astrocytoma, but are rare in pediatric extracranial solid tumors, including neuroblastoma. Here we report the case of an infant with Stage MS neuroblastoma with a rare BRAF V600E mutation and subsequent progression to Stage M disease.