PURPOSE :  Recurrent giant cell tumors refractory to various treatment modalities are challenging dilemmas for the most experienced practitioner.  We sought to present and report on a patient with osteoglophonic dwarfism and an aggressive giant cell tumor of the maxilla and mandible unresponsive to all treatment modalities. 

METHODS:  The records of a 6 year old girl with a recalcitrant giant cell tumor diagnosed at 10 months of age were reviewed.  We outline the course and treatment of traditional giant cell tumors, as well as the experimental treatments attempted in this patient, including the original use of antiangiogenic therapy with Bevacizumab for this type of tumor.  An extensive review of the literature is presented. 

RESULTS :  Tumor growth persisted despite multiple attempts at surgical debulking, embolization, and adjuvant treatment including chemotherapy, immunotherapy and the use of an antiangiogenic monoclonal antibody. 

CONCLUSION :  This is the first reported case of an aggressive giant cell tumor of the jaw unresponsive to any available treatments.  Further research needs to be conducted into the exact mechanisms of osteoglophonic dwarfism and its association with aggressive tumor growth.  Additionally, a further understanding of current anti-angiogenic therapies and their role in the treatment of giant cell tumors is warranted.