Here we present the case of a 26-year-old man in whom peripheral primitive neuroectodermal tumour of chest wall origin invaded the left lung with regional lymph node metastasis. He underwent initial resection of the left chest wall tumour with combined left lower lobectomy, left S5 segmentectomy, and lymph node dissection in order to facilitate a definitive diagnosis and also to obviate the risk of fatal bleeding due to tumour invasion of the pulmonary artery. Histological examination of the resected sample revealed small round cell proliferation with neural differentiation, and confirmed lymph node involvement within the left lower lobe. EWS/FLI-1 fusion gene transcripts were detected by the reverse-transcription polymerase-chain reaction. Diagnosis of peripheral primitive neuroectodermal tumour was confirmed. After surgery, combination chemotherapy with cyclophosphamide, vincristine, doxorubicin, ifosphamide, and etoposide was given. Five years after resection, the patient remains alive and well, with no signs of recurrence.