Adenoid cystic carcinoma (AdCC) is a rare cancer originating from secretory glands with unknown aetiology. It is one of the most dominant malignant salivary tumours (MST). However, it can arise in other areas of the head and neck region and in secretory glands outside this area. It occurs at all ages, but is more frequent between 50-70 years of age and more common in females than in males. The symptoms of AdCC are generally unspecific and the clinical diagnosis of AdCC maybe challenging, partially due to its heterogenous histopathology and indolent growth. Moreover, there is a lack of good prognostic markers, and due to its rarity, it is difficult to predict which therapeutic methods are the most optimal for each patient, especially since very late recurrences occur. This review presents some major characteristics of AdCC and some current treatments for this disease.Head and neck cancer (HNC) is the 6 th most common cancer worldwide with around 900,000 new cases and >400,000 deaths yearly (5, 6). Important risk factors for HNC are smoking, alcohol, opium, betel quid chewing, oral infections, radiation exposure, personal history of HNC and more recently even viral infections, e.g., human papillomavirus (HPV) and Epstein-Barr Virus (EBV). However, for some HNC e.g., malignant salivary gland tumours (MST), including adenoid cystic carcinoma (AdCC), risk factors are still mainly unknown (1-5, 7). Recently, in many Western countries, due to a decrease in smoking, the spectra of various HNCs have changed. We are seeing a steady decrease in smoking related HNC but instead a parallel rise in HPV associated head and neck cancer has been disclosed (8-10).