We report the first known South African case of fibroblastic rheumatism, a rare dermatoarthropathy. Our patient presented with the typical clinical features of a sudden-onset, erosive polyarthritis with cutaneous nodules and sclerodactyly. Significant functional loss occurred within a period of 3 to 4 months. X-rays of the hands showed a single erosion, whereas magnetic resonance imaging showed further erosions as well as soft tissue and synovial enhancement. The unique histologic findings of fibroblastic proliferation, thickened collagen, and dermal fibrosis confirmed the diagnosis of fibroblastic rheumatism. Our patient was treated with a combination of methotrexate and oral prednisolone with subsequent resolution of her synovitis/arthritis and no further progression of her sclerodactyly and associated functional loss. The course of fibroblastic rheumatism is known to vary and although multiple therapeutic options have been tried, the question remains whether any of the therapies alters the natural course of the disease. However, considering the probable role of lymphocytes and fibrogenic cytokines, an increased awareness with early diagnosis and treatment in the initial inflammatory stage may prevent the development of incapacitating joint sequelae.