To identify the clinical and pathological features of natural killer-like T-cell lymphoma/leukemia.The characteristics of natural killer-like T-cell lymphoma/leukemia was discussed with report a new case and review of literatures.A 16-year-old girl was referred to our hospital because of fever and disseminated cutaneous herpes and ulcer. Atypical lymphoid cells surrounded the dermal vessels with a CD3(+), CD8(+), CD4(-), CD5(-), CD10(-), CD19(-), CD57(-), CD56(+), perforin(+), granzyme B(+) immunophenotype and rearranged T-cell receptor-gamma gene implicated natural killer-like T cell origin. She was treated with prednisone and for several months. Then the patient developed progressive spleen enlargement with overt leukemia, which led to her eventual death.Natural killer-like T-cell lymphoma/leukemia is a rare disease with distinctive clinical, histopathologic, and immuno phenotypic characteristics. Current treatment modalities are ineffective for most of the patients.