Ewing's sarcoma and primitive neuroectodermal tumor are rare tumors of childhood, both considered to be small round blue cell tumors of childhood. Recent advances have been made regarding the histogenesis and biology of these tumors. Current thinking links Ewing's sarcoma and primitive neuroectodermal tumor by a shared chromosomal translocation and by evidence of neuroectodermal origin. Advances in chemotherapy have resulted in the cure of most patients presenting with Ewing's sarcoma. This review briefly outlines the year's literature on the histogenesis, biology, and treatment of this tumor.