Abstract: Objective To analyze the clinical pathologic features of tonsil pleomorphic rhabdomyosarcoma．Methods A case of tonsil pleomorphic rhabdomyosarcoma was described and the related literatures were reviewed． Results The histological appearances of tonsil pleomorphic rhabdomyosareoma was similar to that of the other parts of body．Immunohistochemical staining for Myoglobin was positive． Conclusion There are less strap and tadpole-shaped cells and predominantly round cells in pleomorphic rhabdomyosarcomas，the latter playing an important role in the diagnosis．The immunohistochemical staining can increase the accuracy of diagnosis．It should be differentiated from other tumors originated from tonsil mesenehyme cells．And the subgroups of the rhabdomyosareoma should be differentiated，in other to choose the right therapy．