This paper discusses the orofacial manifestations of children diagnosed with severe microcephaly according to the World Health Organization. Microcephaly is a clinical condition made at or after birth that characterizes a significant reduction in head circumference, which may present craniofacial, neurological, and oral alterations as a result of this malformation. As skull growth is linked to facial growth, can patients with microcephaly have structural and functional impairments? Will these possible changes be expressed before skeletal maturity? These issues are discussed through the presentation of two relevant clinical reports of children diagnosed with severe microcephaly with similar clinical facial and occlusion patterns.. Studies along these lines should be conducted with a view to future attempts to minimize possible effects of this malformation on the craniofacial complex with preventive and interceptive therapeuticals in these individuals.