Introduction: Several cases of long bone giant cell tumor have been reported in the literature. We report the case of a patient with a giant cell tumor in the distal ulna. This is very unusual, with a reported incidence of 0.45 to 6%. Case presentation: A 25-year-old female presented with a painful swelling of the right wrist. After performing a detailed clinical examination and investigations, a diagnosis of distal ulna giant cell tumor was made. The tumor was treated with an En bloc Resection, biopsy and stabilization of wrist using tri cortical bone graft. Conclusions: This tumor may have a good prognosis if it is diagnosed early and radically treated. It is important to be aware of atypical cancer localizations in order to perform a proper diagnosis.