ABSTRACT
Primary idiopathic chylopericardium is a rare clinical entity characterised by the collection of chyle within the pericardial cavity without a definitive cause. This case report describes the history, physical examination, evaluation, diagnosis and treatment of a 19-year-old boy with primary idiopathic chylopericardium. Radiological findings and biochemical analysis of the pericardial fluid following pericardiocentesis sustained this diagnosis. Initial conservative management failed, and the patient was surgically treated subsequently. He recovered well postoperatively and remained asymptomatic thereafter. Primary idiopathic chylopericardium is a rare pathology with very few cases reported till date, and the symptoms are commonly due to cardiac compression. Computed tomography of the chest and bipedal lymphoscintigraphy are considered the standard methods for accurate diagnosis, and in cases of failed medical treatment, open and thoracoscopic thoracic duct ligation with pericardiectomy have been described as the best surgical options.