Chylothorax is a rare condition characterized by an abnormal occurrence of chyle into the pleura. In children, it can be a potential life-threatening disorder. The optimal therapeutic strategy remains controversial between conservative and surgical treatment, and there is no clear evidence when to operate. Most pediatric reports in literature say that conservative treatment should be long. However some surgical procedures have proved to be beneficial if performed after a shorter delay even in congenital forms. Several surgical options are possible through different approaches: drainage, pleural abrasion, pleurectomy, thoracic duct ligation or repair, which can be done by thoracotomy or by thoracoscopy. All surgeons using minimally invasive procedures have reported good experiences in small series, shortening the previously reported delay for healing.