A case of T-cell lymphoma of granular lymphocyte (GL) morphology and CD3+CD4+CD56+ phenotype is reported. The lymphoma originated in the subcutaneous tisse of the right thigh and subsequently infiltrated into various organs including the lymph nodes, peripheral blood, bone marrow, skin, lungs, liver, spleen, and peritoneal cavity, and the patient finally developed a hemophagocytic syndrome. The lymphoma cells were positive for CD2, CD3, CD4, and CD56, and negative for CD8, CD16, CD20, and CD57. An identical clonal rearrangement of the T-cell receptor beta chain genes was detected in cells from a lymph node, peripheral blood, and ascites fluid, indicating the monoclonal proliferation of T cells in such organs. This is a T-cell GL lymphoma, expressing the unusual phenotype of CD3+CD4+CD8-CD16-CD56+, which has not been previously reported. The development of hemophagocytic syndrome has also to our knowledge not previously been reported in T-cell GL lymphoma.