A patient with intestinal lymphangiectasia secondary to constructive pericarditis is reported. He presented with retarded growth, asymmetrical and generalized oedema, low serum proteins, lymphopenia and low serum gammaglobulin. The diagnosis was based on the observation of dilated lacteals of the villi in a biopsy of intestinal mucosa and by the augmented excretion of Cr-51 labelled proteins in the feccs. Echocardiographic findings were compatible with constrictive pericarditis. The diagnosis was confirmed at surgery, which was follovcd by clinical improvement.