Objective To summarize the clinical manifestations, diagnosis and treatment of severe primary graft dysfunction ( PGD grade 3 ) in early stage after lung transplantation. Methods From September 2002 to December 2010, there were 10 patients with severe PGD ( grade 3) in early stage after lung transplantation ( LTx) in 100 patients with end-stage lung disease underwent LTx in Wuxi People’s Hospital. In which there were 2 cases with chronic obstructive pulmonary disease, 4 with idiopathic pulmonary fibrosis,1 case with lung tuberculosis, 1 case with silicosis, 2 cases with bronchiectasis. There were 7 patients with single LTx [ 3 cases with extracorporeal membrane oxygenation ( ECMO) support] and 3 patients with bilateral LTx ( 1 case with ECMO support) . Results The surgical procedures of these 10 patients were successful, however severe PGD occurred on 1-5 days after operation. 4 cases died of respiratory failure with negative fluid balance and mechanical ventilation support, and 2 cases recovered. 4 cases underwent ECMO support, in which 2 cases successfully weaned from ECMO and discharged from hospital, others died of multiple organ failure.Conclusions Severe PGD is one of the fatal early complication after lung transplantation. Early diagnosis and treatment are very important to improve the perioperative mortality rate.