Background: Myxoma is the most common tumor of the heart that can cause embolism, obstruction, and cardiac failure, but rarely causes chylothorax. We herein report a case of chylothorax caused by left atrial myxoma, which responded to diuretic therapy and was subsequently cured by resecting cardiac myxoma.Case presentation: This is a case of a 63-year-old male with symptoms of cardiac insufficiency who was diagnosed with a left atrial myxoma. The patient also had a massive pleural effusion on the right side, which was diagnosed as chylothorax by Sudan III staining and the content of triglyceride. The pleural effusion disappeared and the symptoms of heart failure were relieved after draining the effusion with a fine thoracic drainage tube, but pleural effusion and cardiac insufficiency symptoms relapsed soon. Although diuretic treatment can improve the symptoms and reduced the amount of chylous fluid preoperatively, the chylous pleural effusion was eventually cured by surgical removal of the tumor, and no recurrence of the tumor and chylothorax was found in the 6-month follow-up.Conclusions: Chylothorax is rare comorbidity of cardiac myxoma. Large myxoma can cause congestive heart failure and lead to disfunction of chylous reflux, resulting in chylothorax, which can be cured by resection of the primary tumor.