Pulmonary interstitial glycogenosis (PIG) is a non lethal disease, reported in neonates with respiratory distress syndrome developed shortly after birth [193, 194] . Very few cases are described so far but it seems to have a male preponderance [195] . The histological hallmark of pulmonary interstitial glycogenosis is the accumulation of monoparticulate glycogen in the interstitial cells on lung biopsy. It is thought to represent a maturation defect of interstitial cells that leads them to accumulate glycogen within their cytoplasm [8, 196] . It is discussed that PIG could meet "chronic pneumonitis in infancy" as this remains a generalized term [87] . As well, PIG could be considered as a premature lung disease, but more than half of published cases were in term infants [195, 197, 198] . The long term consequences in these infants need to be ascertained.