Ascites is a late and uncommon symptom of Chronic Granulomatous Disease (CGD) of childhood following liver damage with portal hypertension due to hepatic granulomatosis. The Authors describe two patients with CGD, in whom ascites was the main symptom in the first case and the initial symptom in the second case. Ascites in these cases was not due to liver fibrosis but it was a consequence of primary bacterial peritonitis.