Chylothorax is defined as an accumulation of chyle in the pleural space caused by disruption of the thoracic duct or one of its major divisions. Chyle has a high content of triglycerides. The odorless fluid is turbid and milky due to the presence of fat containing particles, the chylomicrons. The etiology of chylothorax can be divided into four major categories: tumor, trauma, idiopathic and miscellaneous. Although chylothorax is uncommon, it is a serious and potentially hazardous disorder. Loss of chyle leads to metabolic disturbances, malnutrition and immunodeficiency. Treatment consists of treatment of the underlying disease, conservative treatment (medium chain triglyceride diet, parenteral nutrition) or surgical intervention. Appropriate timing of surgical intervention is essential. Since the ligation of the thoracic duct can be performed during thoracoscopy, this minimal interventional technique is the procedure of choice when conservative treatment fails.