Vulvar localisation of schistosomiasis is a rare presentation. We report a case of a woman of 20 years old hospitalised for a vulvar mass of six months. That mass progressively increased in volume and was tender. It was accompanied by frequency and dysuria. The patient had a history of swimming in fresh water and hematuria when she was eight years old. Initial clinical examination found a good general state, there was a mass involving the clitoris and the small lips. This painless mass had a cauliflower appearance and was soft with a large implantation. Pathology exam of the mass revealed a vulvar schistosomiasis with an important amount of living eggs. A tumour removal with a plasty of small lips was performed. Additionally, praziquantel was administered orally. Vulvar localisation of schistosomiasis might suggest a malignant tumour. Only pathological examination can assess the diagnosis.