A 52 year old man developed an extraskeletal mesenchymal chondrosarcoma (ESMC) arising from the pleura. Clinically, the tumour mimicked a mesothelioma. Fine needle biopsy was consistent with the diagnosis of sarcomatoid mesothelioma. Histological examination of multiple tumour samples, supported by immunohistochemical characterization, made it possible to correctly diagnose extraskeletal mesenchymal chondrosarcoma.