A newly described immediate complication after percutaneous pulmonary valvuloplasty isacute lung injury. Here we report a case of fatal acute lung injury after pulmonary valvuloplasty.The patient was a 26-year-old woman, referred to a general hospital with the diagnosis of livercirrhosis. In her work-ups severe pulmonary stenosis was detected and so a decision was madeto relieve the valve stenosis. Despite the procedural success, the patient developed severe dyspneaand desaturation a few hours later and died within 3 days due to shock state. Although thedefinition, incidence or severity of acute lung injury after pulmonary balloon valvuloplasty is notyet clear, this is as far as we know the first mortality reported in literature. This presentation inour patient should prompt clinicians to consider a more aggressive approach at the first sight ofthis previously considered innocent complication.