Background: Congenital Chylothorax (CC) is a rare condition, which is defined as an accumulation of the chyle in the pleural cavity; moreover, it is associated with significant morbidities, including respiratory distress, malnutrition, immunodeficiency, and infections.Case Report: The diagnosis of chylothorax was made upon count cell analysis of the pleural fluid with >= 80% lymphocytes detected before birth or within 28 days after birth. In this study, we presented five cases of CC infants. They were discharged from our tertiary center at Hedi Chaker Hospital, Sfax, Tunisia, from January 2010 to December 2018. There were three males and two females. Prenatal diagnosis was made in four cases. There were four full-term newborns and one near-term of 36 weeks. Pleural effusion was on the right side in three cases, on the left side in one case, and bilateral in one case. Four cases required mechanical ventilation. Somatostatin was indicated in one case. The treatment was successful in four cases. One case presented a dysmorphic syndrome and died of pneumothorax.Conclusion: The treatment of CC is based on conservative management. Somatostatin or its analog octreotide is considered an adjunctive treatment of CC. However, the refractory cases are treated with chemical pleurodesis or surgical treatment. We propose an algorithm for the treatment of CC.