The case history of a patient with primary liposarcoma of the humerus associated with a schwannoma of the radial nerve in the arm is described. Ultrastructurally, the neoplasm showed multiple and varied lipid droplets, dilated RER [rough endoplasmic reticulum], mitochondria with electron-dense matrix and microfilaments as the most prominent features. Based on these ultrastructural observations, liposarcoma of the bone apparently is similar to pleomorphic liposarcoma arising in the soft tissues. This is the 1st ultrastructural study of a primary liposarcoma of the bone and the 1st reported association of this tumor with a schwannoma. The possible pathogenetic relationship between both tumors remains unknown.