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Mantle cell lymphoma (MCL) is an aggressive type of malignant lymphoma with short median survival despite conventional therapy. We describe the unusual case of a 66-year-old man with a chronic skin rash which preceded the occurrence of an indolent MCL by 2 years. Repeated skin biopsies did not show involvement by the lymphoma. Short therapy with rituximab resulted in the complete and lasting resolution of the cutaneous lesions.