Vulvar lichen sclerosus (VLS) is an uncommon skin disease that is complicated frequently by the loss of normal vulvar architecture and less commonly by vulvar squamous neoplasia including vulvar intraepithelial neoplasia (VIN) and invasive squamous cell carcinoma (SCC). Incidence of vulvar SCC among patients with VLS were reported to be relatively high as about 5%. But, these patients may be unfamiliar to dermatologists because most of them visit gynecologic clinic. We present a case of 39-year-old woman with 30-year history of vulvar pruritus. Six years ago, she was diagnosed with VLS by biopsy from the ivory-white plaque with severe pruritus and pain in the vulvar region. She had been treated over the years with topical tacrolimus 0.1% ointment and oral antihistamines. One year ago, she revisited our clinic due to occurrence of new solitary erythematous nodule and ulcerative patch on the pre-existing lesion. Histopathologic findings for the nodular lesion showed atypical keratinocytes invading the dermis, multiple mitotic figures, keratin pearls. Finally, SCC arising from VLS was diagnosed based on clinicopathologic features. Although SCC arising in VLS has rarely been reported in Korean dermatologic literature, it’s incidence is relatively high and increases according to disease period. Therefore, we suggest that long-term follow-up of the patient with VLS should be essential due to the risk of developing SCC.