Massive ascites present at birth without generalized edema is a rare manifestation with a grave prognosis. Three large groups of underlying anatomical abnormalities have been implicated in the pathogenesis of this disorder. Baghdassarian,1 in a recent review, lists: (a) intestinal anomalies, especially perforation of the bowel secondary to malformation; (b) portohepatic abnormalities with obstruction of the portal circulation; and (c) anomalies of the lower urinary tract. A fourth group of miscellaneous causes includes cases of chylous ascites. Of approximately 165 reported cases in all four groups,