The authors describe a tase of a 57-year-old woman presenting with an enlargement of her right 4th toe without a bone involvement on X-ray examination. Punch biopsy findings included nodules separated by collagenous septae formed by middle sized polygonal mononuclear and foam cell positive for anti-PGM positive (macrophage marker) with an admixture of B and T lymphocytes. The diagnosis of a giant cell tumor of a tendon Sheath was suspected. Because of the tumor extension the amputation of the whole digit had to be performed. Histological examination revealed additional features consisting of scattered multinucleated giant cells, xanthoma cells, cleftlike spaces and hemosiderin pigment which confirmed the diagnosis. Bone invasion was not present. Six months after the surgical intervention there esere no signs of tumor recurrency.

        Key words: giant cell tumor of tendon Sheath-localized type (pigmented villonodular tenosynovitis) - histopathology - immunohistochemistry