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This report describes 16 cases of the recently recognized anaplastic large cell Ki-1 lymphoma. The disease showed a male:female ratio of 2.2:1 and a bimodal age distribution with peaks in the third and eighth decades. The clinical presentation was highly variable, with lymph node, skin, bone and gastrointestinal tract being the most commonly affected sites. The lymph nodes usually showed subtotal or sinusoidal involvement, and parenchymal fibrosis was common. The large neoplastic cells were almost invariably admixed with many reactive small lymphocytes, histiocytes and/or neutrophils. Two cytological types could be delineated: type I (pale cell, four cases) consisted of large polygonal cells with distinct pink-staining cell membrane and pale cytoplasm and pleomorphic nuclei showing marked chromatin clearing; and type II (basophilic cell, 12 cases) consisted of round or oval cells with basophilic cytoplasm and/or paranuclear pale hof, pleomorphic nuclei often reniform or lobulated and with frequent multinucleated wreath-like and Reed-Sternberg-like cells. Immunohistochemical studies showed that nine cases (56.3%) exhibited a T-cell phenotype, three cases (18.8%) each exhibited a B-cell or null-cell phenotype, while one case exhibited both T- and B-cell markers. Cutaneous involvement at presentation was associated with a favourable outcome, and spontaneous regression was common. For patients with non-cutaneous presentation, the prognosis was relatively good for young patients treated with aggressive chemotherapy, but was grave for old patients.