An 80-year-old female patient G7P4, presented with a 6-month history of palpable mass in the right region of the vulva, associated with occasional pain. On physical examination, we identified a solid mass on the lower third of the vulva, between the labia majora and labia minora that projected towards the vaginal introitus. It was approximately 4 cm, partially adhered to deep planes and slightly painful to touch. The rest of the exam was normal. Transvaginal and transperineal ultrasound identified a solid well defined mobile mass with regular borders, and mixed echogenicity with some calcifications that had slight posterior acoustic shadows, in the area that anatomically corresponds to the Bartholin gland in the right vulva. Its measurements were 37x35x33mm and volume of 16cm3. Additionally, it presented an increased vascular pattern Doppler score 4. A three-dimensional (3D) high-definition power Doppler ultrasonographic reconstruction was performed in order to improve the vascular characterisation of the mass. The magnetic resonance imaging confirmed a solid well-defined lesion of 40x28x27 mm in the right vulva, without infiltrating adjacent structures, and intense enhancement with contrast medium. She underwent vulvovaginoscopy and superficial mucosal biopsy which had non-specific findings and was negative for a malignant lesion. Wide local excision of the mass and vaginoplasty were carried out without complications. The pathology report evidenced a vulvar adenoid cystic carcinoma (ACC) with positive margins and perineural invasion, classified as at least pT1b (TNM, AJCC 8th Edition). Currently the patient is awaiting further clinical staging in order to start adjuvant therapy. Vulvar ACC is a rare neoplasia (<1%) and its ultrasound preoperative evaluation has not been established yet. Therefore, we present the sonographic characteristics, from transvaginal-transperineal technique and 3D high-definition power Doppler reconstruction, of a vulvar ACC.