Background: Malignant vulvar lesions arise from premalignant vulvar intraepithelial neoplasm (VIN) and occur only rarely (3–4 % of all malignancies of the female genital organs), especially in the menopause. They are associated with human papillomavirus infection. If the change is found sufficiently early and if it is properly treated, the prognosis is favourable. Methods and patients: We present a case of a 60-year-old multiparous postmenopausal woman with recurrence of the vulvar cancer. In May 1995 vulvar biopsy revealed VIN III. In February 2006 she was examined at the Oncology Institute because of expansive lesions which covered the entire area of external genitals from clitoris to the anus. On 7th March, 2006 vulvectomy with broad safety margins and right-sided lymphadenectomy were made. Histological di- agnosis revealed a well-differentiated invasive squamous cell carcinoma (FIGO II, grade I); postoperative radiotherapy followed. In February 2007, suspicious lesions reoccurred in the external genital area, the smear was negative. In December 2007 histological diagnosis of recurrent squamous cancer was made. On 7th October, 2008 the patient was admitted to the Department of Gynecology, University Medical Centre, Ljubljana. Abdominal computed tomography showed a widespread vulvar cancer, T4 in appearance, suspicious lymph nodes in the pre-sacral area and suspicious left inguinal lymph node. Metastases to the liver were not confirmed. On 28th October, 2008 total Brunschwig operation was performed (pelvic exenteration of the bladder, uterus, pelvic lymph nodes, rectum and radical vulvectomy with resection of both ramus ossis pubis), anus praeter and transposition of skin-muscle flap (m. rectus abdominis). The interdisciplinary operation, which lasted 9 hours and blood loss was 6 litres, involved gynaecologists, a urologist, a plastic surgeon, and an oncology surgeon. The histological diagnosis of a 27 × 24 cm large preparation was a moderately differenti- ated planocellular carcinoma, which was totally removed. The operation was followed by prolonged post-operative physical and mental rehabilitation, during which beside wound infections with various microbes also Klebsiella pneumoniae ESBL sepsis, and later Candida sp. caused sepsis developed. This led to prescription of a long lasting treatment with systemic antibiotics and antimycotics. On 2nd February, 2009 re-necrectomy and plastic surgery of the sacral region were made. During a 143-day hospitalization, the patient’s depression was regularly managed by clinical psychologist. On discharge from hospital she was in good physical and mental condition, did not have problems with digestion, took care of the anus praeter herself, the reconstruction of the defect was aesthetically good and functional. On the follow-up examination 3 months later she was in good physical and mental condition, and returned to her workplace. Conclusions: This case report is intended to remind us that regular follow-up examinations of patients after completed treatment is of utmost importance; in case of recurrence of suspicious changes they require to be treated by appropriate and most qualified medical experts.