Accounting for less than 5% of all vulvar malignancies, primary carcinoma of the Bartholin gland is a rare pathology. Adenocarcinomas or squamous cell carcinomas are the most common histologic types. Diagnosis is made in many symptomatic cases after resection of what was thought to be a Bartholin’s cyst or abcess. Surgical options, depending of the extension of the disease, include radical local excision, radical hemivulvectomy and radical vulvectomy with or without inguinofemoral and pelvic lymphodissection. Adjuvant radiotherapy, in selected cases, may decrease the rate of recurrence.