We report a case of recurrent phyllodes tumor of the vulva in a 39-year-old woman.

Phyllodes tumor of the vulva is extremely rare with only 6 cases reported in the literature.

Primary clear cell carcinoid tumors of the vulva need to be considered in the differential diagnosis of vulvar masses with clear cell features.

These findings provide further evidence that phyllodes tumors of the vulva might derive from mammary-like glands in the labium majus or from ectopic breast tissue.

Two patients were treated with simple vulvar tumor excision at other medical institutions without adjuvant therapy.

The vulvar tumor size and inguinal lymph nodes metastasis will influence the prognosis, with pathologic differentiation and surgical margin status being the probable prognostic factors.