Cystic fibrosis (CF) is a genetically inherited autosomal recessive disease characterized by dehydration of the airway surface liquid and impaired mucociliary clearance leading to chronic respiratory infections, bronchiectasis and ultimately death from respiratory causes. Common pulmonary related complications resulting from bronchiectasis include pneumothorax and hemoptysis. A rare, seldom reported complication is acute pericarditis complicating cystic fibrosis. Our review of the literature identified only a single case of culture-proven purulent pericarditis attributed to Pseudomonas aeruginosa in a patient receiving systemic corticosteroids. A second reported case of acute pericarditis in a CF patient was attributed to Mycoplasma pneumoniae which is a known cause of infectious-related pericarditis independent of CF. Both cases required open pericardial drainage with 1 fatality. Here we present a case of cardiac tamponade from a fibrinous pericardial effusion in a 21-year-old female as a complication of CF related parenchymal lung disease. To our knowledge, this is the first reported case of a non-infectious parapneumonic pericardial effusion causing near fatal cardiac tamponade as a complication of CF lung disease.