With the exception of moderately differentiated neuroendocrine carcinoma of the larynx, neuroendocrine carcinomas are very rare head and neck malignancies. We report a case of a small cell neuroendocrine carcinoma in the petrous apex of the temporal bone. This unusual site for this tumour has not yet been reported in the literature. We confirmed the location and extent of the primary tumour by positron-emission tomography scan. The final histopathological diagnosis was of small cell carcinoma, and this was confirmed by immunohistochemistry. We treated this patient with surgery followed by radiotherapy and chemotherapy. After the treatment was completed there was subtotal remission of the tumour, with no distant metastases.