Chylous ascites is rare, accounting for less than 1% of cases. An appropriate and stepwise approach to its diagnosis and management is of key importance. To review the current diagnostic approach and management of chylous ascites. A literature search was conducted using PubMed using the key words 'chylous', 'ascites', 'cirrhosis', 'pathophysiology', 'nutritional therapy', 'paracentesis", "transjugular intrahepatic portosystemic shunt" and "TIPSS'. Only articles in English were included. Chylous ascites is caused by the traumatic or obstructive disruption of the lymphatic system that leads to extravasation of thoracic or intestinal lymph into the abdominal space and the accumulation of a milky fluid rich in triglycerides. The most common causes are malignancy, cirrhosis and trauma after abdominal surgery. This condition can lead to chyle depletion, which results in nutritional, immunologic and metabolic deficiencies. An ascitic triglyceride concentration above 200 mg/dL is consistent with chylous ascites. Treatment is based on management of the underlying cause and nutritional support. Chylous ascites is mostly due to malignancy and cirrhosis in adults, and congenital lymphatic disorders in children. Treatment with nutritional optimization and management of the underlying etiology are the cornerstones of therapy. When conservative measures fail, other interventions such as octreotide/somatostatin analogues, surgical ligation, embolization and transjugular intrahepatic portosystemic shunt in patients with cirrhosis can be considered.