A 48-year-old man presented with a 4-day history of fever and 10-year history of papulovesicles on the face, neck, trunk and limbs which had been aggravated 10 days prior to the presentation.Skin biopsy showed a dermal infiltration of numerous small- to medium-sized atypical lymphocytes, which was mainly located around blood vessels or appendages, with the involvement of subcutaneous fat tissue and destruction of blood vessels. The infiltrating atypical cells stained positive for CD45RO, CD8, CD56, T-cell intracellular antigen-1, granzyme B, Epstein-Barr virus-encoded small nuclear RNAs (EBER), but negative for CD20, CD79a, CD3, CD4 or CD30. Cytoplasmic CD3ε was also observed in these cells. Laboratory examinations on admission revealed a progressive decrease in peripheral erythrocytes, white cells and platelets, persistent increase in serum aminotransferase and bilirubin, and decline in serum fibrinogen and hypertriglyceridemia. The B-mode ultrasound of the abdomen showed hepatosplenomegaly. Based on the above findings,the diagnosis was made as extranodal nasal type NK/T-cell lymphoma of skin complicated by hemophagocytic syndrome.