Spontaneous chylothorax associated with primary lymphedema and a lymphangioma malformation

Spontaneous chylothorax associated with primary lymphedema is an exceedingly rare clinical entity. We report a case of chylothorax in a 38-year-old woman with a history of primary lymphedema of the right leg and an inguinal lymphangioma. Pathophysiology, diagnosis, treatment, as well as literature review are presented.