Although rare, chordomas may arise from extra-axial locations, which are mostly of soft tissue origin.3,7,8 Besides soft tissue, there are a few reports of chordomas that originate in the lungs, which is currently the only reported visceral organ where extraosseous chordomas occur.9,10 Interestingly, Kikuchi et al11 recently reported 2 cases of incidental notochordal tumors of the lung that were indicative of BNCTs, as evidenced by brachyury expression. The gene discussed is TBX1; the disease is chordoma.