Pleomorphic rhabdomyosarcoma of the diaphragm represents fewer than 0.0001% of all cancers. One case is reported, and a total of eight cases to date are reviewed. It is an adult disease of both sexes, the lowest reported age being 14 years. The tumor presents at a late stage, and the average symptom-death interval is only a few months. Early diagnosis and wide excision are essential along with radiotherapy and chemotherapy before any survival--much less worthwhile survival--can be hoped for. There is evidence of host immune response against rhabdomyosarcomas. Immunotherapy, therefore, should be considered. For assessment of the biological behavior of the tumor and the treatment, standardization of staging and grading are essential, although there is some evidence that the prognosis of primary and recurrent tumors treated radically are similar.