Background Extramammary Paget’s disease is a rare condition, and the vulva is a common site for it to occur. Despite this, there is a paucity of literature on Paget’s disease of the vulva (VPD). A Cochrane meta-analysis could not draw any conclusions on interventions in VPD. Our aim was to review our practice and improve further management of VPD in our center.

Methods We reviewed all the cases presented to Leeds Gynaecological Oncology Centre between 1988 and 2016. All cases identified in this interval were followed up until April 2016. All case notes and electronic patient data were retrieved to collate the data.

Results We identified 18 cases of VPD. The median age at presentation was 76.9 years. Primary surgery was used in 18 cases. Eight patients had wide local excision with graft reconstruction. Ten women had wide local excision with primary reconstruction. Margins were negative in 27% of the excisions. Sixty percent of patients with clear surgical margins had a recurrence, and 69% of patients with positive margins had a recurrence; there was no statistical difference between the 2 groups for recurrence (P > 0.05). Fifty-eight percent of patients who had recurrence had coexisting malignancy. Logistic regression showed no correlation of recurrence rates due to either age, margin status, or coexisting malignancies.

Conclusions Paget’s disease of the vulva is a rare condition. Our experience indicates that most cases may be amenable to surgical treatment at first presentation. Negative margin status does not reduce the chance of recurrence, and hence patients should be under follow-up for life. The benefit of radical surgery in the absence of reduced recurrences, based on margin status, is questionable. Radiotherapy and imiquimod are options for extensive lesions or recurrent settings. Coexisting malignancies are associated with VPD.