We herein report a case of Richters syndrome(RS) that was discovered following an investigation of a skin lesion. RS represents an acute transformation of chronic lymphocytic leukemia(CLL) to highly malignant large cell lymphoma such as diffuse large B-cell lymphoma(DLBCL). RS is characterixed by abrupt B-symptoms, rapidly progressive lymphadenopathy, hepatosplenomegaly, frequent extranodal involvement, hypercalcemia and an unfavorable clinical course. The patient is a 31-year-old male with skin lesions on his face. He had been previously diagnosed with CLL. At his presentation, CLL and DLBCL manifestly co-existed. Our multistage analysis suggested that DLBCL and CLL might arise from a different B-cell clone. He showed a poor clinical response to systemic chemotherapy. (Ann Dermatol 10(4) 274-280, 1998).