ABSTRACT. Extraskeletal myxoid chondrosarcoma was found in a five-month-old male Irish setter dog. At necropsy, the largest mass, measuring 15 × 13 × 13 cm, was found in the right caudal lobe of the lung, and metastatic small masses were observed in multiple organs. Microscopically, the tumor comprised mainly atypical mesenchymal cells and myxoid stroma, which stained positively with Alcian blue. Immunohistochemically, the tumor cells stained positively for vimentin, S-100 protein, neuron-specific enolase, calretinin, and chromogranin A. Ultrastructurally, the cytoplasm of the tumor cells was comprised abundant rough endoplasmic reticulum, mitochondria, Golgi complex, free ribosomes and short irregular microvillous processes extending from the cytoplasm. Based on these pathological findings, this tumor was diagnosed as extraskeletal myxoid chondrosarcoma. Primary chondrosarcomas arise from existing normal cartilage and perichondrium at sites such as the pelvis, nasal cavity, sternum, and ribs, and less often in long bones. Extraskeletal chondrosarcoma was first described as an entity in 1953 by Stout and Verner [23]. Characteristically, in most reported cases, extraskeletal chondrosarcoma exhibits less aggressive behaviour than its skeletal counterpart [5,21,24].A series of extraskeletal myxoid chondrosarcoma in man was described in 1972 by Enzinger and Shiraki [5]. Almost all the tumors in this series affected adults (mean age 49 years), and the only child was a 13-year-old girl. In animals, reports of extraskeletal chondrosarcoma are rare, and most cases have been described as a single mass in adult or old dogs [1,9,13,19,22]. In this case report, we describe an extraskeletal myxoid chondrosarcoma with systemic metastasis in a five-month-old dog.A five-month-old male Irish setter dog was referred to our veterinary hospital with a history of anorexia, dyspnea, diarrhea, and exophthalmos of the left eye. The dog had been fully vaccinated, and there was no previous medical or surgical history. Under sedation, radiographs of the thoracic cavity were taken and confirmed the presence of a large, poorly defined mass lying within the right caudal lobe of lung. Approximately three-fourths of the thoracic cavity was occupied by the pulmonary mass, and systemic metastasis was confirmed by abdominal radiographs. Because of the poor prognosis, the dog was euthanized. A necropsy examination was performed.Tissue samples from all major organs and masses were fixed in 10% buffered formalin, embedded in paraffin wax, and sectioned. All sections were stained with hematoxylin and eosin (HE), and selected sections were prepared for staining for Alcian blue (pH 2.5), periodic acid-Schiff (PAS), and immunohistochemistry. The following primary antibodies were used: S-100 protein (Nichirei Biosciences, Tokyo, Japan), neuron-specific enolase (NSE, Nichirei), vimentin (Nichirei), cytokeratin (CK) AE1/AE3 (Nichirei), α-smooth muscle actin (SMA, Dako-Japan, Kyoto, Japan), desmin (Dako-Japan), calretinin (Zymed Laboratories, Carlsbad, CA, ...