Neuroblastoma is the most common neoplasm in the first year of life and almost always arises from the adrenal glands or the sympathetic nervous system chain. We present an original case of an isolated forearm neuroblastoma in a 17-month-old child. The case was not treated with adjuvant chemotherapy after surgical resection because we interpreted it as a peripheral, INSS-stage 1, favorable histology neuroblastoma. Up to date the child remains in complete remission 4 years after diagnosis. Management of peripheral skeletal muscle neuroblastoma is discussed together with a review of the literature.