A-19-year old male patient complained of shortness of breath. Aspiration of the pleural fluid revealed chylothorax. Right chest tube was inserted. His ABG showed hypoxaemia with relative hypercarbia. He underwent right thoracotomy and thoracic duct ligation under general anaesthesia and double lumen endobroncheal intubation. During surgery he lost 1.5 L of blood and 4 L chyle. He was transferred to the SICU intubated and on mechanical ventilation. On the subsequent days chyle leak was reduced to a minimum of 10 ml/hr. On the 9th postoperative day the patient was extubated. He was receiving TPN 2600 kcal/day. He was transferred to the normal floor on the 15th day. After 7 day he was readmitted, his chest showed severe lung fibrosis and consolidation. His ABG showed severe hypercarbia (PaCO2 = 126 mmHg). The patient was intubated. His condition deteriorated and he was considered for lung transplantation. No donor was available. Later he arrested and died. Anaesthesia and surgical management of spontaneous chylothorax is challenging. The mortality rate is high.