The development of ascites indicates a pathological imbalance between the production and resorption of intraperitoneal fluid. The appearance and composition of ascites are variable, based on the underlying pathophysiology. Most commonly, ascites develops in the setting of decompensated cirrhosis, peritoneal infection, carcinomatosis, congestive heart failure or a combination (mixed ascites). The diagnosis can be difficult in some patients. Management options for ascites from decompensated liver disease focus on low-sodium diets and diuretics supplemented by large-volume paracentesis, transvenous intrahepatic portosystemic shunts and liver transplantation. The development of refractory ascites, hepatic hydrothorax, hyponatraemia or hepatorenal syndrome presents unique challenges to the provider and the patient. In some of these patients, therapy with liver transplantation will be the only viable therapeutic option. The diagnosis of infectious ascites, such as tuberculosis, and carcinomatous ascites remain diagnostic and therapeutic challenges for the clinician.