The authors report a case of acquired lymphangioma circumscriptum (LC), localised in the right labium majus of vulva and characterised by periodic symptoms for 8 years; however, 3 months after the right major labiectomy, the same symptoms developed in both the left labium majus and the right labium minus. To the best of our knowledge, this is the first case of acquired LC with this type of localisation and symptoms without any known aetiologic factor.