Objectives: The aim of this article was to evaluate the clinical and pathologic characteristics, therapy, and prognostic factors of vulvar sweat gland carcinoma. Materials: Clinical and pathologic data for 12 patients with vulvar sweat gland carcinoma treated at our institution from January 1958 to April 2009 were retrospectively analyzed. Of the 12 cases, 7 cases were vulvar sweat gland carcinoma, 3 cases were vulvar Paget disease with underlying sweat gland adenocarcinoma, 1 case was vulvar apocrine adenocarcinoma, and 1 case was adenoid cystic carcinoma of the vulvar sweat gland. Two patients were treated with simple vulvar tumor excision at other medical institutions without adjuvant therapy. Among the other 10 patients, 6 underwent radical vulvectomy; 3, wide local excision of the vulva; and 1, a simple vulvectomy. For 5 of the 12 patients, bilateral or unilateral inguinal lymph nodes excision and biopsy were performed. For 1 patient with bulky inguinal lymph nodes, only a biopsy was performed, and the patient received radiotherapy after vulvar surgery. Results: A follow-up for 11 patients was conducted until death or April 1, 2009. Five of the 11 patients had recurrences after primary treatment. For 2 of these patients, recurrence was local 6 and 48 months after treatment. For 3 patients, distant metastasis was found 18, 5, and 31 months after surgery at our institution. Five of 11 patients died, 1 of whom died of irrelevant disease and 4 of tumor progression. The total survival periods of the 4 patients who died of tumor progression were 24, 36, 44, and 203 months. The other 6 patients have survived for more than 5 years without local failure. In total, there are 7 patients who have survived for 5 years or more. Conclusions: Vulvar sweat gland carcinoma is a very rare entity. Surgery is the primary treatment modality, and the function of radiotherapy and chemotherapy is uncertain. The vulvar tumor size and inguinal lymph nodes metastasis will influence the prognosis, with pathologic differentiation and surgical margin status being the probable prognostics factors. Copyright © 2010 by IGCS and ESGO.