Malignant peripheral nerve sheath tumours (MPNSTs) may occur in any peripheral nerve. They are often found in the chest wall and the posterior mediastinum. On the other hand, primary pulmonary MPNST is extremely rare, and surgically treated cases have been reported. Here, we present 3 cases of primary MPNST originating from the pulmonary parenchyma who underwent surgery in our institution. We discuss the possible clinical and pathological associations in the view of the literature.