Neuroblastoma has been bescribed as a malignant tumor that arises from sympathetic ganglion cells and, as such, may occur in any area of the body where these cells are found. It is the most common soft tissue malignant neoplasm in childhood. Although metastasis of neuroblastomas to the head and neck from more common primary sites in the abdomen, pelvis, or thorax is common, to our knowledge, well-documented primary head and neck lesions are quite rare. The prognosis of invasive neuroblastoma of the head and neck is poor, with rapid and widespread metastasis being the rule. However, some success has been reported with early and massive therapy that involves surgery, chemotherapy, and radiation therapy. The authors experienced a case of neuroblastoma of the neck. A 14-year old female, whose chief complaint was progressive difficulty in respiration for four to two weeks, was admitted to the hospital. We report this case with review of the literatures.