Background: Neuroblastoma is the most common extra-cranial paediatric solid tumour. This embryonal neoplasm is derived from neural crest cells and usually forms a mass in the adrenal medulla (80%) or mediastinum (15%). Primary neuroblastoma of the mandible is extremely rare. We report a case of a primary neuroblastoma arising in the mandible of a six year old boy, his management and a review of the literature.

Clinical case: The child presented with a painless mass in the mandible. Clinical examination and investigations confirmed the diagnosis of a primary neuroblastoma of the mandible. Intra-abdominal and thoracic pathology were excluded. The patient was treated with neoadjuvant chemotherapy followed by surgery involving segmental resection of the mandible, bilateral selective neck dissection, reconstruction with a fibular free flap and further chemotherapy. He subsequently underwent dental rehabilitation with an implant retained prosthesis.

Conclusion: This is a rare, complex case requiring a multidisciplinary team approach. Future problems are anticipated with mandibular growth and the resulting changes in occlusion. These issues will be discussed together with a review of the current literature on its possible aetiology and management.

Conflict of interest: None declared.