Neuroblastoma is the most common extracranial neoplasm among paediatric patients, which arises from ectodermal neural crest cells. The most frequent primary site of neuroblastoma arises from retroperitoneum, specifically in the adrenal medulla. We present a rare case of adrenal neuroblastoma metastasis to maxillary sinus in a 20 month-old boy presenting with right maxillary swelling for the past 2 months. Computed tomography (CT) scan of paranasal sinus demonstrated right maxillary mass. A biopsy performed via a sublabial approach, surprisingly revealed neuroblastoma. Subsequent further workup depicted a huge right adrenal tumour. He was diagnosed with adrenal neuroblastoma stage 4 and treated with chemotherapy and surgical resection.