Palmar fasciitis and polyarthritis (PFPA) is an uncommon syndrome characterized by progressive and extensive rheumatic disease. It has usually been associated with neoplasms, most commonly in elderly patients, preceding or accompanying the diagnosis of malignancy. We report a case of a 25-year-old patient with severe deforming PFPA preceding the diagnosis of carcinoma of the ovary. The histopathological findings of diffuse fibrosis, connective tissue proliferation and vasculitis with C3 and IgM deposits in subcutaneous tissue and synovia may further clarify the possible pathogenesis of this unique syndrome.