Chylous ascites is the accumulation of a milk-like peritoneal fluid rich in triglycerides, due to the presence of thoracic or intestinal lymph in the abdominal cavity. It develops when there is disruption of the lymphatic system due to traumatic injury or obstruction (from benign or malignant causes). In Western countries abdominal malignancy and cirrhosis account for over two thirds of all cases. In contrast, infectious etiologies such as tuberculosis are responsible for the majority of cases in developing countries. Other causes of chylous ascites include congenital, inflammatory, postoperative, traumatic, and miscellaneous disorders. Congenital abnormalities of the lymphatic system and trauma should be considered as etiologic factors in children. Diagnosis of chylous ascites can be readily made. The presence of a milky and creamy ascitic fluid with a triglyceride content above 200 mg/dL makes the diagnosis of chylous ascites. In a cirrhotic patient, unless there is a strong suspicion of malignancy, unnecessary, expensive and invasive diagnostic modalities to rule out a malignant process should be avoided. The management of patients with chylous ascites is based on identifying and treating the underlying cause. Nutritional support with a low-fat diet and medium-chain triglyceride supplementation is of paramount importance. Therapeutic paracentesis and diuretics are recommended in patients with underlying cirrhosis.