In this article, we retrospectively reported the successful diagnosis and treatment for a 88-year-old primary biliary cirrhosis patient with chylothorax. He initially presented with fever, cough, hemoptysis and shortness of breath, then he was found with pleural effusion and diagnosed with real chylothorax after admission. After exclusion of some common reasons, such as tumor, tuberculosis and so on, he was considered to be diagnosed as chylothorax secondary to primary biliary cirrhosis with the aids of abdominal CT scanning and positive anti-mitochondrial antibody M2. Surgical treatment was given in the case that conservative treatment of internal medicine was not effective. After surgical treatment, the patient recovered well and chylothorax was not recurrent in multiple chest X-ray examinations. This report aims to promote the physician’s awareness for chylothorax in the elderly, so that the early diagnosis and general treatment is performed to prolong the patient’s survival time.