BACKGROUND: Chondrosarcoma is a malignant tumor of chondrogenic origin and the mesenchymal type is a very rare finding. Mesenchymal chondrosarcoma tends to develop mostly in the skeleton but may also occur as a primary tumor in periosteal nervous and muscular tissues, the anterior cerebral falx, meninges, brain, maxillary sinus, eyelid, thyroid, pleura and mediastinum, while in the abdomen the most frequent locations are the kidney, retroperitoneum and even the perineum and the anogenital area. Apparently, the only splenic mesenchymal chondrosarcoma in the literature occurred in a dog. METHODS AND STUDY DESIGN: Our paper reports the case of a patient who had a diagnosis of mesenchymal chondrosarcoma of the spleen. Results. We adopted surgery as the main therapeutic procedure without achieving complete recovery but preserving a good quality of life for our patient, minimizing the repercussions of the disease on her working and relational life. CONCLUSIONS: The absence of important or invalidating symptoms and the persistence of good general conditions before and after each surgical operation encouraged us to adopt the surgical option as the most rational.
