Primitive malignant neoplasms affecting the distal third of the tibia are altogether rare, and their treatment is considerably controversial. The authors describe the diagnostic procedure and a particular surgical strategy of limb salvage in a case of malignant fibrous histiocytoma located at the distal third of the tibia, in particular pointing out the difficulties in restoring the continuity of the skeletal tissues and of the muscle, and with a review of the relevant literature.