AbstractThe association of systemic sclerosis (SSc) and non-Hodgkins lymphoma (NHL) is a rare event whose pathogenesis remains to be clarified. We describe a case of NHL in a patient with SSc, in whom the NHL was particular in terms of clinical presentation (primitive muscle and bone involvement) and histologic type (large multilobated B-cell variant). The lymphoma was clinically aggressive but responded well to therapy. Surveillance for the development of atypical NHL is suggested in SSc cases.