AbstractIntroduction: Adenoid cystic is an infrequent malignancy of the salivary glands. Some of its unique features include slow growth, the tendency for perineural invasion, local recurrence, and the ability to metastasize. The mobile tongue's adenoid cystic carcinoma (ACC) is a rare reported case in the literature. Report: We report a rare case of adenoid cystic carcinoma, in a 55-year-old female arising in the dorsal and lateral aspect of tongue. Histopathology after complete excision of the mass confirmed the diagnosis of adenoid cystic carcinoma. There has been no evidence of disease following excision with tumour-free margins. Discussion: The pathophysiology of ACC is an understudied area due to the condition's rarity. Chromosomal abnormality and dysregulated MYB oncoprotein have to be thought to play a key role in the pathophysiology and proliferation of ACC. Conclusions: Adenoid cystic carcinoma of the tongueis exceedingly rare but should be considered in the differential diagnosis in patients with tongue swelling. These tumours are malignant and treatment requires surgical excision and neck dissectionfollowed by adjuvant radiotherapy.