Primary cutaneous carcinomas rarely show heterologous malignant mesenchymal differentiation. We report 11 cases of sarcomatoid basal cell carcinoma (BCC) with osteosarcomatous differentiation. The patients (7 men and 4 women) ranged in age from 61 to 92 years (median 75 y). The tumors presented as exophytic nodules (0.3 to 7 cm) on the head (n=6), upper limb (n=3), and lower limb (n=2). All lesions were completely excised. Seven patients were alive without evidence of disease (follow-up interval 5 to 24 mo) and 1 patient died of unrelated causes at 7 months without evidence of disease. On histology, the tumors were dermal in location with 2 cases showing focal subcutaneous involvement. Ten tumors were well-circumscribed and 1 tumor showed focally infiltrative edges. Ten tumors revealed conventional BCC associated with varying proportions of osteosarcomatous and undifferentiated sarcomatous stroma. Transition from neoplastic epithelial to mesenchymal cells was seen in 8 cases. One case showed a purely osteoclastic giant cell rich malignant mesenchyme, interpreted as representing early stages of osteosarcomatous transformation. Previously unreported in sarcomatoid BCC, the mesenchymal component of another two cases displayed predominant malignant giant cell tumor like areas and 1 further case disclosed areas reminiscent of telangiectatic osteosarcoma. Pancytokeratins (AE1/3 and MNF116) and smooth muscle actin stained occasional undifferentiated sarcomatous cells in 2 and 3 tumors, respectively. MNF116 and EMA were focally positive in osteosarcomatous tumor cells of 1 case. Although the follow-up interval is short, our data suggest an excellent prognosis for polypoid and exophytic sarcomatoid BCC after complete surgical resection.