Desmoid tumors are benign neoplasms with high rates of recurrence. A case of huge desmoid tumor of the intrathoracic and intraabdominal space is presented. The patient was treated with resection, which involved hepatic left lobectomy and diaphragmatic resection and partial pericardiectorny and wedge resection of left lower lobe of lung. The resulting defect over the pericarium and diaphragm was reconstructed by bovine pericardium and Marlex mesh. Prevention of presumed local recurrence of desmoid tumors requires wide excision margin.