Abstract Tracheal tumors are uncommon, comprising only 0.2% of respiratory tract malignancies, with the majority of these being squamous cell carcinoma and adenoid cystic carcinoma. Tracheal chondrosarcomas are a rare cause, with only thirty-four cases described in literature between 1959 and 2020. Although often asymptomatic, nonspecific symptoms including dyspnea, nonproductive cough, and wheezing contribute towards misdiagnosis with diseases such as asthma or chronic obstructive pulmonary disease (COPD). Delay in diagnosis can potentially lead to complete tracheal obstruction and airway compromise. Treatment of choice is surgical resection, with overall 10-year survival to be around 95%. Although recurrence rates are minimal when treated with open surgical resection with negative margins, post-surgical surveillance is recommended. Use of extracorporeal membrane oxygenation (ECMO) during surgery offers an alternative to endotracheal intubation, potentially making complete tumor resection easier. A 56-year-old male never smoker with past medical history of hereditary hemochromatosis, requiring phlebotomy, severe obstructive sleep apnea status post right upper airway nerve stimulator placement, and a history of left vocal cord granuloma that presented to an outpatient pulmonology clinic for chronic cough and exertional dyspnea. Initially, the patient was started on a proton pump inhibitor and nasal corticosteroid, without improvement in symptoms. He subsequently underwent pulmonary function testing, which showed flow volume loop suspicious for variable extrathoracic airway obstruction. Chest computed tomography (CT) scan was significant for a 2.2 cm soft tissue lesion in the left upper mediastinum with protrusion through the tracheal wall. PET scan did not show significant FDG avidity. A 1.5 centimeters lesion situated about 5 centimeters above the carina was found on rigid bronchoscopy and biopsies were negative for malignancy. Patient subsequently underwent video-assisted thoracoscopic surgery (VATS) robotic-assisted tracheal resection on Veno-venous ECMO. Final pathology was consistent with G1 chondrosarcoma, with negative margins. On follow up, patient's respiratory symptoms have resolved and periodic surveillance with repeat chest CTs is planned. This unique case offers an important reminder of a potential cause of upper airway obstruction, not only highlighting the potential of pulmonary function testing to aid in early detection, but also the successful use of ECMO in surgical resection.