After describing a condition involving both lungs uniformly or only a portion of a single lobe with multiorgan abnormalities by McMahon in 1947 (MacMahon 1948), in 1981, Janney and her colleagues (Janney et al. 1981) described congenital alveolar capillary dysplasia for the first time. Alveolar capillary dysplasia with misalignment of pulmonary veins (ACDMPV) is one of the poorly known, rare, and lethal developmental disorders of the lung that affects infants and neonates (Ma et al. 2017). Ninety-five percent of ACDMPV patients are full-term (Bishop et al. 2011) and have no problem with birth weight or Apgar score (Slot et al. 2018). In most cases, the onset of respiratory distress and severe pulmonary hypertension (PH) unresponsive to treatment occurs within 24 hours after birth (Bishop et al. 2011; Ma et al. 2017; Slot et al. 2018). However, late-onset cases have been reported (Sen et al. 2014). In classic types of ACDMPV, the mortality rate is almost 100% (Slot et al. 2018), and they die because of respiratory failure in the first weeks of life (Ma et al. 2017). But in atypical cases, some survivors have been reported. There is no specific geographic pattern, and patients can be found all over the world (Bishop et al. 2011). Reported cases have shown 60% male predominance (Bishop et al. 2011). The incidence is about 1/100,000 (Slot et al. 2018). Estimating the true prevalence of ACDMPV is challenging because the definitive way to diagnose ACDMPV is based on histological findings of lung tissue on biopsy or ante mortem lung biopsy, but neither of these two methods is commonly used when dealing with a very ill or dying newborn. Also, the lung pathology in ACDMPV patients can be underdiagnosed because of multiorgan abnormalities. In addition, some pathologists may have missed the diagnosis of ACDMPV (Bishop et al. 2011).