Ameloblastic carcinoma is a rare odontogenic tumour with histologic features of ameloblastoma but with malignant potential. The majority occur in the mandible, arising de novo, or develop from pre-existing a benign ameloblastoma or odontogenic cysts. They typically present as a rapidly progressing, painful swelling of the face. Maxillary ameloblastic carcinoma is exceedingly rare and when reported, can present in a similar manner to mandibular ameloblastic carcinoma. We present a case of maxillary ameloblastic carcinoma in a 78-year-old man presenting with a 12-month history of unilateral nasal obstruction and frontal headaches. A benign-looking nasal lesion was identified and the patient was diagnosed with nasal polyps and underwent biopsy. However, histopathological examination of the lesion together with radiological imaging pointed towards a diagnosis of ameloblastic carcinoma. The patient was referred to our tertiary skull base centre and underwent successful treatment with radical surgical resection via a midfacial degloving approach. Final pathological examination of the surgical specimen confirmed the diagnosis of maxillary ameloblastic carcinoma. This case demonstrates an unusual presentation of an exceptionally rare malignancy, contributing to the growing number of reported cases in the literature of maxillary ameloblastic carcinoma.