Pericarditis is the most frequent cardiac manifestation of systemic lupus erythematosus (SLE), but pericardial effusion causing tamponade rarely occurs, and it is even less frequent for the pericardial tamponade to be the presenting feature of SLE. We report a case of pericardial tamponade due to SLE with severe hemodynamic involvement as the clinical presentation of the disease. We comment on its clinical course and its rarity in the medical literature.