A 49-year-old man was admitted with high-grade fever, night sweating and cervical lymphadenopathy in September 2005. On examination, both neutrophilia and thrombocytosis were noted in the peripheral blood, a bone marrow examination revealed marked both myeloid and megakaryocytic hyperplasia. The sera obtained at initial presentation showed an elevated levels of granulocyte-colony stimulating factor (G-CSF) and interleukin-6 (IL-6). A pathologic diagnosis of T-cell rich B-cell lymphoma was made based on an inguinal lymph node biopsy. Following treatment with CHOP accompanied by rituximab (R-CHOP), both the neutrophilia and thrombocytosis subsided after 3 courses of R-CHOP, resulting in a complete remission after 4 courses of chemotherapy. Neutrophilia, thrombocytosis and T-cell rich B-cell lymphoma in this patient were considerably ameliorated with chemotherapy. We report here a patient with T-cell rich B-cell lymphoma associated with both neutrophilia and thrombocytosis, suggesting that the lymphoma triggered both myeloid and megakaryocytic hyperplasia.