Dedifferentiated parosteal osteosarcomas are characterized histologically b
y the combination of low-grade fibroblastic osteosarcoma admired with a hig
h-grade component that typically has the appearance of malignant fibrous hi
stiocytoma or osteosarcoma. Herein we report a case of dedifferentiated par
osteal osteosarcoma of the distal femur, in which the high-grade component
consisted of rhabdomyosarcoma. To our knowledge, a rhabdomyosarcomatous com
ponent has not been described previously in a dedifferentiated parosteal os
teosarcoma. The clinical, radiologic, and pathologic features of this rare
type of surface osteosarcoma are described.