Malignancy developing. in extragonadal endometriosis is rare with the major
ity of cases being endometrioid adenocarcinomas. We present a 54-year-old w
oman who developed a clear cell carcinoma in her perineum, 25 years after e
xcision of a perineal endometrioma in an episiotomy scar. Treatment involve
d radiotherapy and surgery, with a view to preserving vaginal function. Whe
re benign extragonadal endometriosis occurs, the potential for malignant tr
ansformation persists even years after primary treatment.