Desmoid tumor is a rare, locally-invasive fibromatosis with a high recurrence rate and non-metastatic features. Here, we reported a 62-year male patient with desmoid tumor in the superior sulcus of left lung, complaining of cough, chest pain, limited-movement, and pain on the left upper arm. We performed extended resection of the tumor, including wedge resection of the left upper lobe, resection and anastomosis of partial left subclavian artery, resection of T1 nerve root, cauterization of adhesive pleura, and resection of the left first and second ribs. After surgery, the patient's symptoms were relived. The duration of hospital stay was 8 d. This is the first reported case of surgical treatment for the superior sulcus desmoid tumor.