Alveolar capillary dysplasia with misaligned pulmonary veins (ACD-MPV) is one of the major cause of neonatal interstitial lung disease causing persistent pulmonary hypertension of the newborn, although literature shows that symptoms can present later in infancy. Two patients were recently diagnosed with ACD at our Institution; we found out that a structured review and description of all the aspects of the disease was lacking. We considered published articles from 2018 to 2024. Final population analyzed: 149 patients, including our 2 cases. From each case report we extrapolated: clinical presentation, radiologic reports, genetic and histopathological features, treatment, and outcome. Pregnancy and birth are usually uneventful. Respiratory failure due to PAH during neonatal period is typical, but later presentation is possible (feeding problems, failure to thrive). Abdominal malformations are associated. Radiological exams rule out other causes of hypoxemia, but a final diagnosis of ACD requires genetic or histological confirmation. The unique curative therapy is lung transplant; intensive support therapy (mechanical ventilation, vasoactive and pulmonary vasodilator drugs) is invariably needed. ECMO is described in 44 patients and should be considered when medical therapy and mechanical ventilation are insufficient. Average survival without lung transplant is 21 months, but it is reduced to 4 months if outliers are excluded. 10 described patients received lung transplant; average survival was 81 months; 6 of them were alive at time of publication. Improvements in intensive support therapy can improve clinical conditions and survival to transplant target weight. Studies on genetic targets are in progress.