We present an extremely rare case of Woringer-Kolopp disease with CD4+/CD8+ imm unohistochemical features in a 27-year-old male patient, which involved the right gluteal region continuing down to the lower thigh. Although the lesions had been present for 15 years, the disease abruptly changed its indolent clinical behavior in the last 3 months and gained local aggressiveness without features of dissemination. The patient was successfully treated with 6 MeV electron-beam radiotherapy and the radiation portal was determined to be the gross lesion plus 1-cm margins around the lesion. Radiation therapy was administered with a hypofractionated dose schedule of 2.8 Gy per fraction in 13 fractions, 5 days a week, to a total of 36.4 Gy. No radiation-induced toxicity was reported during therapy. The patient's follow-up was ordinary with no recurrence after a follow-Lip period of 38 months and the cosmetic result was excellent.