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.