Massive intrapulmonary haemorrhage and haemothorax are uncommon presentations associated with pulmonary sequestration. Here, we describe the case of a 40-year-old man who suffered from high fever and haemoptysis for 1 week before he was admitted to our hospital with a complaint of chest discomfort with shock. Computed tomography revealed that pulmonary sequestration supplied from the coeliac artery with persistent bleeding. The patient underwent right lower lobectomy and an emergent laparotomy for ligation of the aberrant artery. A pulmonary sequestration has a severe complication resulting in shock due to intrapulmonary haemorrhage and haemothorax. Accordingly, early resection of a sequestered lung should be the choice of the treatment in these cases.