Primary muscular involvement is extremely rare in non-Hodgkin's lymphomas. To our knowledge few cases are reported in literature and all of them concern patients with unifocal muscular lymphoid masses usually growing in one of the extremities. Our case-report, instead, regards a 78 years-old woman presenting primary multifocal muscular involvement by extranodal non-Hodgkin's lymphoma (right upper and lower limbs affected at the same time). Therefore, in contrast with the therapeutic approach suggested by other Authors in such neoplasms (radiotherapy or combined radio-chemotherapy), we preferred to administer only chemotherapy. The treatment led to a complete regression of all lymphoid masses. By now the woman is healthy and disease-free as confirmed at the one-year haematological follow-up.