Background: Extraocular sebaceous carcinoma (SC) arising in the vulva is extremely rare that no treatment consensus has been well-defined. Case presentation: We here presented two cases of vulval SC in a 31-year-old and a 62-year-old woman, respectively. Radical wide local excision was performed with free margin and they received no postoperative adjuvant therapy. No evidence of disease was detected after follow-ups for 12 months and 49 months, respectively. A comprehensive literature review of vulval SC was further conducted and other ten cases were included. The mean age was 55.9 years, nine patients were diagnosed with FIGO stage I diseases while the remaining three patients had metastatic lesions at initial diagnosis. Surgery was the mainstay treatment option that 11 (91.7%) underwent surgical resection, of which 5 patients received inguinal lymphadenectomy and 2 patients showed lymph nodes involved. Radiotherapy and chemotherapy were given in 2 and 1 patient, respectively. Two patients experienced recurrence within 1 year after initial therapy. At the final follow-up, ten patients had no evidence of disease, one patient was alive with the disease, and only one died of the disease. Conclusion: Radical wide local excision may be preferred in early-stage vulval SC and utilization of sentinel lymph node sampling should be recommended. Postoperative adjuvant therapy may be spared in patients with negative surgical margin and absence of lymph node involvement. Treatment of vulval SC referring to the guidelines of vulvar cancer should be administered in case of positive margins or metastatic disease.