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Cutaneous γ/δ T-cell lymphoma is an extremely rare and highly aggressive tumor that is often resistant to treatment,such as polychemotherapy and radiotherapy. Due to the complexity of clinical, pathologic, and immunohistochemical features of this disease entity, the physician should perform a careful evaluation; however, treatment should be rapid and aggressive. We present a case of fatal cutaneous γ/δ T-cell lymphoma of a 55-year old woman who died after recurrence with central nerve system metastasis. (Ann Dermatol 23(S1) S100∼S104, 2011)