A 61-year-old man presented with a painful mass overlying the lower left scapula of four months duration. His pain deteriorated the last three weeks and was accompanied by limitation of the range of motion of the left glenohumeral joint. Physical examination revealed a subcutaneous tender uniformly firm mass in the lower left scapular region measuring 4.5 × 4.5 cm in diameter. There were no signs of superficial skin inflammation. The patient had not a previous history of melanoma and/or other skin tumors. Radiological examination showed an osteolytic lesion in the lower left scapula while bone scanning was positive with increased uptake in the same area. CT scans and MRI images of the thorax and shoulder showed a soft tissue mass 45 × 45 mm localized in the lower scapular region between infraspinatus and teres minor muscles with bone invasion of the lower part of the scapula. There were no lung metastases neither axillary nor mediastinal lymph nodes. The open biopsy specimen consisted of a rubbery, homogenous, white tissue with no attachment to the skin, implying deep location of the tumor. There were compact nests and fascicles with large rounded and spindle-shaped cells separated by connective tissue septa. The large rounded cells had pleomorphic nuclei and large amounts of clear cytoplasm while the spindle-shaped cells had palely staining eosinophilic cytoplasm. Mitoses were moderately numerous. There were areas of necrosis and hemorrhage and tumor spreading into the surrounding muscles and scapula. Immunohistochemical stains were performed with negative staining for CD68, actin and desmin antigens and with positive staining for tumor markers S-100 protein, HMB-45, NSE, EMA, cytokeratins and myosin. Histochemical stains for melanin presence were negative. Pathologic findings were compatible with a CCSTA with osseous extension into the lower scapula. After three days a wide excision was carried out and the tumor was removed together with the two thirds of the left scapula and the overlying soft tissues. The remaining bone and soft tissue margins were widely free of tumor. Macroscopic and pathologic examination of the removed mass confirmed the primary diagnosis. Postoperatively the patient started on a chemotherapy protocol (three courses) consisting of ifosfamide, vincristine and epirubicin. The patient had no local recurrences but he developed lung metastases after 5 months. Despite chemotherapy, he died 10 months later with progressive lung disease.