Congenital chylothorax (CCT) is a rare condition which is characterized by an accumulation of lymphatic fluid in the pleural space and exposes the newborn to respiratory distress and losing of proteins, coagulation factors and immunoglobulins. These cases are liable to have sepsis and high mortality rate. We report a case of a female fetus in Gaza delivered at 36 weeks gestational age diagnosed with CCT. The antenatal ultrasonography showed right sided significant pleural effusion and at birth; she had severe respiratory distress. The baby was intubated at birth and right-sided chest tube drain inserted to drain the pleural fluid which was chylus. The case responded partially to intravenous (IV) octreotide and sildenafil. Chylus stopped completely after 2 days of treatment with octreotide and medium chains triglyceride (MCT) oil-based formula feeding. Conclusion: A female newborn diagnosed with right-sided pleural effusion by antenatal scan was confirmed to have congenital chylothorax postnataly. The infant responded to IV octreotide and MCT based formula feeding.