Abstract
Adenoid cystic carcinoma (ACC) is an unusual slow growing salivary gland malignancy with higher chances of recurrence. It is characterized by the proliferation of ductal (luminal) and myoepithelial cells in cribriform, tubular, solid, and cystic forms. Standard treatment, including surgery with postoperative radiation therapy, has attained reasonable local control rates, but distant metastases do not allow any improvement in the survival rate. We present a case of a 50-year-old female diagnosed with ACC involving almost the whole of the upper lip, with an aim to highlight its histologic evolution from it being clinically seen as severely ulcerated and necrotised upper lip mimicking a case of squamous cell carcinoma and its prognosis.