摘要: Background Chylothorax is defined as abnormal accumulation of lymphatic fluid in the pleural space secondary to leakage from thoracic duct or one of its main tributaries. Congenital idiopathic chylothorax is the most common form of pleural effusion in neonates. Conservative therapy is the mainstay of treatment. An alternative therapy is Octreotide, which is a long-acting somatostatin analog that can reduce lymphatic fluid production. It has been used for the treatment of chylothorx in children, but it is used as a new strategy for the treatment of chylothorax in neonates. Case Report We herein report a case of idiopathic unilateral chylothorax in a 3-day-old Iranian male neonate who was treated successfully with conservative therapy, octreotide and regimen therapy with medium-chain triglyceride (MCT)-enriched formula. Conclusion Chylothorax may be suspected in patients with pleural effusions. Pleural fluid triglyceride measurement remains the recommended diagnostic method in chylothorax