Recessive dystrophic epidermolysis bullosa of Hallopeau-Siemens (RDEB-HS) is a rare genetic disorder characterized by trauma-induced blisters, milia, acral pseudosyndactyly, and scarring. RDEB-HS patients present with a distinct pattern of oral involvement consisting of microstomia, ankyloglossia, vestibule obliteration and dental caries. In this review, we describe the orodental manifestations of RDEB-HS and present our experience in a cohort of six new cases of RDEB-HS in children aged 6-10 years, documenting the presence of microstomia, ankyloglossia and vestibule obliteration in childhood. We also show that compared with unaffected control children, RDEB-HS subjects have a greater risk of developing high caries indices with early onset, both for permanent or deciduous teeth, and a worse oral hygiene index (scored as OHI). Tooth malpositions and the cross-bite relationship between maxilla and mandible could play a major role in promoting these events. We propose that dental management of RDEB-HS subjects should commence as soon as tooth eruption begins.