ABSTRACT
Ewing's Sarcoma of bone (ESB) is a rare primary malignant tumor of bone, belonging to Ewing's Sarcoma Family of Tumors (EFT) and are neuro-ectodermal in origin. These tumors are characterized histopathologically as small round blue cell tumors (SRBCT) containing cytoplasmic glycogen, cytogenetically by a (t:11;22) or (q:24;12) translocation and molecularly by the presence of EWS and FLI1 fusion transcripts. ESB is primarily a pediatric tumor, uncommon in the Asian population, affecting the axial skeleton and rarely the jaw bones. ESB poses a diagnostic challenge as it shares many features with other malignant tumors whose managements are substantially different. We present the clinical, radiographic histopathological and immuno histochemical features of ESB involving the left rhinomaxillary complex in a young Indian male. We also discuss the differential diagnosis and current treatment modalities in management of ESB.