Regional odontodysplasia is a non-hereditary development dental anomaly involving epithelial and mesenchymal-derived dental tissues. The condition affects both primary and permanent teeth. Clinically, affected teeth are hypoplastic, soft upon probing, have a yellow-brown discoloration and present high susceptibility to caries. Radiographically, the teeth show enlarged pulp chambers, open apices and no clear differentiation between enamel and dentin. The reduced radiopacity of the enamel and dentin gave rise to the term ghost teeth. We present the case of a three-year-old boy diagnosed with regional odontodysplasia involving more than one quadrant, showing facial asymmetry and missing primary and permanent teeth.