Rhabdomyosarcoma (RMS) represents a malignant tumor of skeletal muscle cells arising from rhabdomyoblasts. RMS represents the most common soft tissue sarcoma in children. In adults it is uncommon and accounts for less than 1% of all malignant solid tumors. While treatment protocols are well known for children, there is no standardized regimen in adults. This is one reason, why the outcome in adults is worse than in children. We present the case of a 59-year-old female patient with pleomorphic rhabdomyosarcoma (PRMS) infiltrating the thoracic spine. Multimodality treatment was performed including en-bloc resection, adjuvant multidrug chemotherapy and radiation beam therapy. The patient was tumor free and had no relapse within 6 month follow-up.