ABSTRACT
Adenoid cystic carcinoma (ACC) is a relatively rare, slow-growing low-grade malignant salivary gland tumour with high recurrence. It accounts for 1% of all malignant tumour of the oral and maxillofacial region. A high incidence of cases occurs between the fifth and sixth decades of life. Surgical excision with required radiotherapy, chemotherapy, and combined therapy are possible treatment choices. In this present case, a 36-year-old male patient was diagnosed with ACC on the upper lip. The clinical presentation of this lesion resembled mucocele. There are only a few cases reported of ACC involving the lip. Complete surgical removal was done under local anaesthesia, and post-operative healing was uneventful. After 6 months of follow-up, no sign of recurrence was observed.