We present a 73-year-old female with an enlarged mass in the right parotid gland. Fine-needle aspiration cytology suggested pleomorphic adenoma. Diagnostic imaging revealed that the tumour had a well-defined margin arising from the deep lobe of the parotid gland. A total parotidectomy with preservation of the facial nerve was performed. The final histopathological diagnosis including immunohistochemical studies was basal cell adenocarcinoma, which is a recently defined entity and a rare epithelial neoplasm. No sign of local recurrence or metastasis 24 months postoperatively has been observed.