Introduction: Adenoid cystic carcinoma (ACC) of the nasal cavity is one of the rarest malignant entities reported in the literature with the known incidence of up to 5%. It commonly poses a diagnostic challenge due to lack of diagnostic suspicion index because of its rarity and especially when it is associated with a long history of benignity. Adenoid cystic carcinoma is a tumor of salivary glands even though it is not frequent. Moreover, it can rarely occur in other body parts, such as nose, paranasal sinuses, lungs, uterine cervix, and many others. Case Report: We present a case of an 18-yearold boy with a diagnosis of ACC of the nasal cavity. The patient had a 10-year history of long-standing chronic inflammatory condition presenting as rhinitis. However, after a period of two years later following the initial diagnosis, the tumor became aggressive and it presented with ulceration, causing erosion of the nasal bone as well as auto-exenteration of the right eye. Conclusion: Adenoid cystic carcinoma involving the nose and other paranasal sites ought to be deemed as a tumor with sometimes long-standing history of benignity which needs high index of suspicion for being able to detect it in order to improve outcome of the patients.