This report describes a patient who presented with pancoast syndrome, secondary to spindle cell sarcoma of the lung. A 56-year man presented with dyspnea, engorged neck veins and bilateral upper limb pitting edema. The patient also had ptosis and miosis in the right eye. Right ulnar nerve palsy with atrophy of hand muscles was seen. His chest X-ray showed bilateral pleural effusion with an opacity involving the apex of the right lung along with mediastinal widening. Echocardiography revealed a pericardial effusion which was drained. The patient's CTscan of chest strongly suspected a malignant mass in right upper lobe with extensive mediastinal lymphadenopathy, pleural metastases and pericardial involvement. He was started on oxygen inhalation, dexamethasone, and clopidogrel. Bronchoscopic biopsy confirmed the diagnosis of spindle cell sarcoma. Meanwhile, he was advised radiotherapy. The tumour was not amenable to surgery. Spindle cell sarcoma is a rare connective tissue tumor that replicates rapidly. To the best of the authors' knowledge, it is hereby reported the first case of spindle cell sarcoma of the lung presenting as Pancoast syndrome.