An updated review of adamantinoma of the appendicular skeleton now provides 195 well-documented cases from the world literature. An additional five new cases are added. Statistically, the tumor remains unusually prevalent in the tibia, but all other major limb bones have been involved, and involvement of several short bones rarely has been reported. The neoplasm is more commonly found in males, but higher earlier age incidence is found in females. The frequent history of preceding trauma may indeed be important in tumor formation. The histogenesis of the tumor is now considered to be epithelial in origin by ultrastructural and immunohistochemical methods. A high incidence of recurrence or metastases is found with inadequate cancer surgery. Known mortalities have indicated severe metastatic disease by aggressive-appearing cells. Previously, early amputation had provided good results, but wide excision or segmental resection with grafting techniques are equally successful. The recent work with allograft replacement of a widely excised segment has shown good early results.