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Background: Primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma is an extremely rare type of cytotoxic lymphoma with increased tendency of spreading to extranodal sites, poor response to conventional therapies for classic CD4+ cutaneous T cell lymphomas, and an unfavorable prognosis. Case report:We present the case of a 40 year old male patient who was referred to our clinic for generalized ulcerated, escharotic, infiltrated papules and plaques and painful ulcerations covered by white deposits on the soft and hard palate of 18 months duration. The unique clinical and histopathological features had posed great diagnostic and therapeutic difficulties. The patient had first been diagnosed with lymphomatoid papulosis, and later with T cell lymphoma and treated with methotrexate, oral retinoids and phototherapy without clinical benefit. During hospitalization in our clinic, the diagnosis of primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma was established and treatment with cyclophosphamide and dexamethasone, associated with local radiation therapy was instituted. Although the cutaneous and oral lesions improved during the first 3 months of treatment, the condition gradually worsened afterwards. Conclusions: Due to the paucity of literature data, frequent atypical presentations, aggressive behavior, and lack of optimal treatment strategies, primary cutaneous aggressive epidermotropic CD8+ T cell lymphoma continues to represent a diagnostic and therapeutic challenge. Further case reports that describe clinical experience and therapeutic outcomes would help clarify its definition, classification and guide the management in this very aggressive cutaneous lymphoma. [ABSTRACT FROM AUTHOR]