A 39 year old woman presented with a slowly growing painless swelling in the 4th intermetatarsal space in the right foot.The resection was done under the primary diagnosis of a ganglioncyst. Surprisingly, the histology gave evidence of an extraskeletal myxoid chondrosarcoma. A comprehensive analysis of the literature on these rare tumors in the extremities revealed that there is a predilection for middle-aged men (male:female ratio, approximately 2:1), especially in the lower extremity. The history of the presented case is typical with a slowly growing painless softtissue mass with varying degrees of radiopacity. The in situ appearance was that of an encapsulated lobulated tumor with no obvious sign of bony erosion or infiltration.