Fibroblastic rheumatism is a rare disease of unknown cause and is characterized by the association of aggressive polyarthritis, sclerodactyly and the presence of cutaneous nodules. The arthritis is rapidly progressive, leading to loss of joint function with flexion contractures of the fingers. The cutaneous lesions are characterized by singular histological findings and consequently their diagnosis is mainly histopathological. Biopsy of a nodule or thickened skin typically reveals increased thickness of collagen fibers and fibroblastic proliferation. The differential diagnosis includes diseases such as rheumatoid arthritis, nodular scleroderma, and multicentric reticulohistiocytosis, among others. Treatment has not yet been consolidated, although most cases have been treated with prednisone and methotrexate with good results. The prognosis is generally good, without functional repercussions, especially if early diagnosis and treatment are provided.