IntroductionNegative pressure pulmonary edema (NPPE) is an uncommon perioperative complication with a potentially fatal outcome. It is most predominant in young healthy men undergoing surgical procedures under general anesthesia. Due to its rare occurrence and uncharacteristic clinical presentation, it poses a potential diagnostic pitfall.AimThe purpose of this article is to present clinical characteristics and management of NPPE.Material and methodsThis paper is based on the available literature and the authors’ experience.Results and discussionClinical presentation of NPPE is uncharacteristic and includes i.e. agitation, tachypnea, tachycardia, cyanosis and pink frothy sputum. Postponed extubation after general anesthesia is believed to be optimal in order to prevent NPPE as it minimizes asynchrony of muscle function reversal and probability of laryngospasm. Differential diagnosis includes and is not limited to pulmonary edema, aspiration pneumonia, anaphylaxis, septic shock, pulmonary embolism or exacerbation of bronchial asthma. Management of NPPE is symptomatic and focuses on symptomatic treatment and maintaining an open airway passage. Endotracheal intubation with low tidal volume ventilation of 6 mL/kg of ideal body weight with a plateau pressure of less than 30 cm H<sub>2</sub>O and high positive end-expiratory pressure (PEEP) may improve patients outcomes.ConclusionsIt is crucial for anesthesiologists to familiarize themselves with this phenomenon for early recognition and proper therapeutic decisions. It should be emphasized that under the highest risk of developing NPPE are young male patients and the most common cause is post-extubation laryngospasm.