Primary Idiopathic Chylopericardium

Primary chylopericardium is a rare and poorly understood entity without established management guidelines. It is thought to be caused by abnormal connections between the lymphatic system and the pericardial space. Medical management includes the placement of a pericardial drain, a low-fat diet, and octreotide to reduce lymphatic drainage. Surgical intervention, including the creation of a pericardial window and thoracic duct embolization, is often required for recurrent cases. We present the case of a young woman who presented with primary chylopericardium refractory to medical management that required surgical intervention. This case highlights the need for the development of management guidelines.