To describe quantitative CT parameters of children with a typical pattern for NEHI and compare them to controls.Eleven patients (7 boys) with NEHI and an available chest CT concordant NEHI were identified. Eleven age-, sex-, height-matched, with CT technique-matching were identified for comparison. An open-source software was used to segment the lung parenchyma into lobes using the fissures. Quantitative parameters such as low attenuation areas, mean lung density, kurtosis, skewness, ventilation heterogeneity, lung mass, and volume were calculated for both controls and cases.Analysis of the lung parenchyma showed that patients with NEHI had a lower mean lung density (-615 HU vs -556 HU, p = 0.03) with higher ventilation heterogeneity (0.23 vs 0.19, p = 0.04), lung mass (232 g vs 146 g, p = 0.01) and volume (595 mL vs 339 mL, p = 0.008) compared to controls. Most lobes followed this trend, except the middle lobe that showed only a higher lung mass (32.9 g vs 19.6 g, p = 0.02) and volume (77.4 vs 46.9, p = 0.005) in patients with NEHI compared to controls.Quantitative CT is a feasible technique in children with a typical pattern for NEHI and is associated with differences in attenuation, ventilation heterogeneity, and lung volume.