Vulval intraepithelial neoplasia (VIN) is the term used for squamous intraepithelial lesions of the vulva, and is classified based on the thickness of epithelium showing dysplasia. The incidence of VIN has nearly doubled over the past two decades and is becoming more frequent in young women aged 20–35 years.¹ Extra mammary Paget’s disease comprises ⩽2% of primary vulval neoplasms, and extra mammary Paget’s disease arising in other sites is even rarer. Incidence of an underlying neoplasm detected in cases of vulval extra mammary Paget’s disease ranges from 0% to 30% in various studies.² However, it is extremely rare to encounter VIN with extra mammary Paget’s disease in the vulva. We present one such rare and interesting association of these two diseases.

A 65-year-old woman presented with whitish plaques on the vulva, along with complaints of pruritus. She had no complaints of any discharge per vaginum, and the …