<b><i>Background and summary:</i></b> Among all vulvar cancers, primary adenoid cystic carcinoma (ACC) of Bartholin’s gland is a very rare tumor characterized by a slow growth, a high local aggressiveness, and a remarkable recurrence rate. Due to its rarity, treatment remains a challenge for oncologists and gynecological surgeons. <b><i>Key message:</i></b> The present paper reports clinical, radiological, and histological features of ACC of Bartholin’s gland and reviews the literature data on the treatment options with a particular focus on the potential role of particle radiation therapy.