Adenoid cystic carcinoma (ACC) of the Bartholin's gland is a rare yet clinically significant malignancy Originating from the Bartholin's gland, situated in the vulvar region, ACC exhibits unique histopathological features and clinical behaviors. In our article, we describe a clinical case involving a 28-year-old female patient who initially presented with a painful swelling on the left labia majora. Initially misdiagnosed as simple Bartholin's gland inflammation, biopsy revealed cystic adenoid carcinoma of the Bartholin gland upon histopathological examination. Radiological evaluation revealed a locally advanced left vulvar lesion with no evidence of distant metastasis. Given the impracticability of surgical intervention, concurrent chemoradiation therapy was administered, resulting in favorable outcomes. ACC has been a diagnostic therapeutic challenge, there is currently no established therapeutic standard. Despite its infrequent occurrence, ACC warrants attention due to its potential for aggressive local invasion, distant metastasis, and propensity for recurrence.