Tumours of the external auditory canal are extremely rare and only 20% of these are of glandular origin. The most frequent histotype is adenoid cystic carcinoma. The rarity of external auditory canal glandular tumours explains the lack of large series reported in the literature and the corresponding large number of case reports from different Authors. Adenoid cystic carcinoma, presenting in the external auditory canal, exhibits the same characteristics as those affecting the major salivary glands, this tumour has an aggressive behaviour characterized by local invasivity and with a metastatic risk of approximately 30%. A rare case of adenoid cystic carcinoma of the external ear is reported. The patient, a 75-year-old male, had right intermittent otorrhea for 6 years. On examination, a vegetating, ulcerated formation which easily bled was found protruding from the right external auditory meatus. Clinical, radiological and pathological features of the tumour are described. A subtotal petrosectomy combined with homolateral elective lymph node neck dissection was performed. Parotid gland, condyle of the mandible and VII cranial nerve were spared since these were free from disease.