Tehran University of Medical Sciences Journal of Family and Reproductive Health 1735-8949 15 1 2021 04 25 70 73 Paola Algeri Department of Obstetrics and Gynaecology, Bolognini Hospital, ASST Bergamo est, Seriate, Bergamo, Italy Rosita Rodella Department of Pathological Anatomy, Bolognini Hospital, ASST Bergamo est, Seriate, Bergamo, Italy Cinzia Manfredini Department of Pathological Anatomy, Bolognini Hospital, ASST Bergamo est, Seriate, Bergamo, Italy Maurizio Algeri Department of Obstetrics and Gynaecology, Locatelli Hospital, ASST Bergamo est, Piario, Bergamo, Italy 2020 12 06 2021 02 08 Objective: Syringoma is a benign adnexal neoplasm of sweat gland, usually presenting as extra-genital lesions, while vulvar localization is rare. Moreover, syringoma is an uncommon vulvar neoplasms. Case report: A 44-year-old woman with previous diagnosis of duodenal gastrointestinal stromal tumour, underwent a local surgical excision for an isolated, painful, vulvar lesion. The specimen was submitted for histological examination. A vulvar syringoma was diagnosed. Conclusion: We describe this case according on its rarity and atypical presentation as well; therefore, vulvar neoplasms encompass many differential diagnoses, among which the incidence rate of syringoma is very low. Although its rarity, syringoma should be included among the differential diagnosis for vulvar neoplasm. https://jfrh.tums.ac.ir/index.php/jfrh/article/view/1626 https://jfrh.tums.ac.ir/index.php/jfrh/article/download/1626/554