Vulvar adenoid cystic carcinoma can be treated by surgery with lymph node dissection, radiotherapy and chemotherapy. Vulvar adenoid cystic carcinoma is a rare and special type of vestibular adenocarcinoma, with common clinical manifestations of painless mass in the posterior part of vulva, which may be accompanied by itching, and with the progression of the disease or secondary infection, skin ulceration occurs, which is easy to be misdiagnosed as vestibular adenoabdominal abscess. The growth of lesions is slow and the course of the disease is long, mostly accompanied by local nerve infiltration. Surgery is the preferred treatment for adenoid cystic carcinoma of the vulva, which can be simple excision, radical excision, with or without lymph node dissection. Radical excision of the vulva with inguinal lymph node dissection is generally recommended. If the tumor is large with metastasis, surgery and radiotherapy can be used to kill the residual tumor cells. Once adenoid cystic carcinoma of the vulva occurs, you should seek medical advice immediately and choose the appropriate treatment under the guidance of your doctor.