A case of giant cell tumor localized in the proximal end of the right tibia, initially treated by simple curettage is described. After 11 years there was local recurrence, confirmed anatomopathologically, observed during wide resection and reconstruction by arthroplasty plus homoplastic grafting. After a few months there were metastatic lesions in the left parietooccipital and right retroorbitary area. Biopsy of the parietooccipital lesion showed giant cells in a sarcomatous stroma. The patient died after several attempts at chemotherapy. Even if only sporadic cases have been described of late recurrences and of extrapulmonary metastases for giant cell tumors, the case presented here is exceptionally rare. The factors that influence recurrence of these tumors and their ability to metastasize is discussed.