Regional odontodysplasia (RO) is an uncommon, nonhereditary, odontogenic developmental disturbance characterized by hypoplasia and hypocalcification of the dental tissues that produce so-called "ghost teeth." This report describes a case of a 2.5-year-old girl who came to the clinic with RO affecting her right maxillary arch. The distinguishing characteristics of this case were the involvement of both the primary and permanent dentitions and the early occurrence of odontogenic abscesses that required the patient's hospitalization. Ultrastructural analysis revealed dental tissue failures that compromised the integrity of the involved teeth, justifying the high susceptibility to caries that was clinically observed. Follow-up was characterized by periodic prosthetic adjustments to maintain the patient's ability to masticate and for social interaction, beyond allowing normal development of her maxillofacial complex. Patients with RO require individualized treatment planning and close follow-up with a multidisciplinary approach.