BACKGROUND: Basosquamous cell carcinoma (BSC) of the vulva is a rare entity with interesting prognostic and therapeutic implications. Currently, there is no definitive treatment due to its low incidence. CASE: A case of recurrent BSC of the vulva treated with unilateral radical vulvectomy and unilateral lymph node dissection is reported. CONCLUSION: BSC is a rare disorder of the vulva. The metastatic potential of this tumor is not fully understood, but likely is intermediate between squamous cell carcinoma and basal cell carcinoma. Local recurrence is common and close follow-up is warranted.