Acantholytic squamous cell carcinoma(ASCC)is a rare histological variant of squamous cell carcinoma,with vulvar ASCC being an uncommon and atypical tissue variant characterized by acantholysis and unique morphological features.Its specific pathogenesis and etiology remain unclear.Given its low incidence,gynecologists generally lack understanding of it,raising the risk of misdiagnosis or missed diagnosis.We report the data of a 79-year-old patient with primary vulvar ASCC,who was admitted to our hospital due to self-reported vulvar pruritus for 5 years.Examination revealed an 8 cmx4 cm red and swollen ulcer on the left vulva with partial white change,and the pathological biopsy showed ASCC.Following 25 times of radical radiotherapy,close follow-up is recommended.And now three months post-radiotherapy,there is no evidence of tumor recurrence or metastasis.There are few research reports on vulvar ASCC both domestically and internationally.This article presents a case of vulvar ASCC in an elderly woman,analyzing its clinical characteristics,pathogenesis,pathogenic factors,differential diagnosis,treatment,and prognosis,in order to provide reference for the clinical diagnosis and treatment of ASCC.