Non-Hodgkin lymphoma of vulva is exceedingly rare and it often poses a diagnostic challenge if their existence is not suspected. We report a patient who has primary cutaneous anaplastic large cell (C-ALCL) with an unusual presentation as a vulvar ulcer. She received a brief course of chemotherapy followed by local irradiation and has remained disease-free more than a year from the time of diagnosis. To our knowledge, primary C-ALCL involving the vulva has never been reported. Despite its typical cutaneous manifestation of C-ALCL, the uncommon presenting site of this entity warrants recognition because of its prognostic and therapeutic implication.