Summary. Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of extranodal non-Hodgkin lymphoma derived from cytotoxic T-cells, usually manifesting by sinusoidal infiltration of spleen, liver and bone marrow. In 1997 World Health Organization classified malignant lymphomas and placed HSTCL among peripheral T-cell neoplasms. The course of the diseases is usually very agressive with a median survival time of 8 to 16 moths despite multiagent chemotherapy. We present a case of a 48-year-old male patient whose initial symptoms were fatigue, weight loss and night sweats, which were followed by splenomegaly and pancytopenia. After clinical examination we suspected him to have HSTCL which was proved pathohistologically upon splenectomy and it is the first case of this lymphoma diagnosed in »Merkur« Clinical Hospital. As a first line of lymphoma therapy we decided to apply FED course (fludarabine, cyclophosphamide, prednisone), being aware of the published poor results the standard CHOP chemotherapy (cyclophosphamide, doxorubicin, vincristine, prednisolone) yields. As far as we know, the results of this chemotherapy course in the therapy of this tumor have never been published. The patient underwent 6 courses of FED therapy, which he tolerated well and was in good clinical condition. Upon the com- pletion of the 6th course of therapy he was diagnosed with lung anaplastic microcellular carcinoma and was treated with 3 course of PE therapy (cisplatin, etoposide).