We report the case of a 23-year-old Japanese man with nasal type natural killer (NK)/T-cell lymphoma and subcutaneous panniculitis-like involvement in the skin. The neoplastic cells possessed the following characteristics of NK cells: expression of cytoplasmic CD3ϵ, CD56, T-cell intracellular antigen-1, azurophilic granules in the cytoplasm, Epstein-Barr virus-encoded small nuclear RNA, and germline configurations of T-cell receptor-β and immunoglobulin heavy chain J genes in the involved tissue. Despite multiple courses of several chemotherapeutic regimens, the cutaneous lesions as well as his general condition deteriorated, and he died of subarachnoidal bleeding. We gathered 14 available reported cases of NK/T-cell lymphoma with subcutaneous panniculitis-like involvement and reviewed the 15 cases, including our patient. They were all of Asian descent. Of the 15 patients (5 men, 10 women), the mean age was 47 years (range, 22-77 years). They had frequent common systemic symptoms: hepatosplenomegaly, weight loss, general weakness, and arthralgia. Twelve of the 15 patients died of disease. The mean duration from diagnosis to death was 9.5 months. NK/T-cell lymphoma with subcutaneous panniculitis-like involvement is associated with a poor prognosis.