We report a case of an infant with spontaneous chylothorax due to the congenital malformation of a small lymph vessel of the chest wall. Conservative therapy with omitting long-chain fatty acids from the diet, fat-free nutrition, total parenteral nutrition and intravenous somatostatin did not result in the decrease of pleural effusion. Thoracic surgical intervention performing thoracic duct ligation and using fibrin sealants was applied after 10 days of unsuccessful conservative therapy, and resulted in the complete recovery of the patient. Our experience support the already existing observations, that in cases where the daily loss of chyle exceeds 100 ml per age years and/or lasts longer than 2 weeks, early surgical intervention is recommended.
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