Although neuroblastoma is a relatively common malignancy of childhood and its dissemination to distant organs is often seen, metastasis to the mandible is rare. A 3-year-old boy which a mandibular soft tissue mass was the initial presenting symptom of disseminated neuroblastoma is reported. The results of biopsy were inconclusive and the differential diagnosis from the imaging studies included lymphoma, soft tissue sarcoma, and osteosarcoma. A metastatic work-up disclosed neuroblastoma of the adrenal gland origin with osseous and bone marrow metastases. Urinary catecolamines were also increased. Regarding the widespread features of the tumor and lack of adequate treatment at this stage of disease, a palliative chemotherapy was conducted, and the patient died one month after starting treatment. This case illustrates that neuroblastoma at a young age, with bone metastases and bone marrow involvement are predictive of the poor outcome of the disease. Therefore, detecting early stage metastasis is one of the essential factors for improving treatment of neuroblastoma patients.