Pindborg tumor in the distal femur.

To describe a case of possible diagnosis of Pindborg tumor on the distal femur. A 32-years-old female patient, a native of Bolivia, resident in Brazil, arrived to this service for tumor research in the right femur. After biopsies and resection of the lesion, the case was referred to analysis and consultancy in the United States. In a report of review by the Pathology Laboratory, it was characterized the histological appearance and immunohistochemical profile were characteristic of Pindborg tumor. Currently, the patient is being followed-up at the Orthopedics and Traumatology Department of this institution, and presents a good evolution. This study presents the case of a patient with a rare tumor that was investigated for an extensive period and through many tests. Pindborg tumor was suggested as a diagnostic hypothesis due to the characteristics and behavior of the neoplastic lesion. Although this lesion is more commonly observed in odontology, the neoplasia was compatible with the diagnosis. Therefore, despite the fact that this tumor has benign characteristics, long-term monitoring is necessary, given the high rate of tumor recurrence.