HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low Platelets) is a thrombotic microangiopathy affecting a minority of women with pre-eclampsia and usually resolves after delivery. The role of plasma exchange in HELLP syndrome has to be defined. Herein is reported a case of a primipara with a class I HELLP syndrome with prevalent pulmonary involvement successfully treated with 8 consecutive plasma exchange plus corticosteroids. In class I HELLP syndrome with cardiopulmonary complications early plasma exchange could be considered a therapeutic option.