RESUMO
Ewing's sarcoma family of tumours include both peripheral primitive neuroectodermal tumour (PNET) and Ewing's sarcoma including both intraosseous and extra osseous sites. Rarest of all is the involvement of liver as primary site, of which only 4 case reports have been published. We report a very rare case of 4 year old male child with primary Ewing's Sarcoma in the liver along with review of literature. The child presented with pain abdomen and low grade fever and hepatomegaly. On putting extensive IHC panel on liver SOL biopsy the small round cells were positive for FLI-1 & CD99 & were immunonegative for OCH, LCA, AFP, WT1, NSE, MYOGENIN, B-catenin & glypican 3. The histopathological and immunohistochemical profile was suggestive of Ewing's Sarcoma. To our knowledge, this is the fifth reported case of Ewing's Sarcoma occurring in liver as primary & first case to be present in a young boy of 4 years age.