Sebaceous epithelioma is a common canine cutaneous neoplasm characterized by a preponderance of basaloid cells with few well-differentiated sebocytes. It is considered a low-grade malignancy, as it may occasionally recur at the excision site; the possibility of lymph node metastasis is anecdotally reported, and distant metastases have never been observed. This case report presents the clinical and pathological features of a sebaceous epithelioma of the upper lip with a highly aggressive behaviour. The patient was a 9-year-old female dachshund that developed local recurrence 11 months after the excision of the primary sebaceous epithelioma and multiple lung and central nervous system metastases 5 months later. The designation epitheliomatous sebaceous carcinoma has been suggested for aggressive sebaceous epitheliomas, although differential criteria are still to be determined.