Bögershausen et al. reported on a KS patient with a KMT2D mutation who presented with neonatal cholestasis with bile duct paucity in addition to the typical clinical features of KS, although their case did not show pathogenic variants in either JAG1 or NOTCH2; they hypothesized that the KMT2D mutation might have affected several key Notch signaling components [24]. This evidence concerns the gene NOTCH2 and cholestasis.