Pseudoxanthoma elasticum (PXE) is an inherited disorder that has both autosomal recessive and autosomal dominant pathways and is characterized by severe malformation of elastic and collagen fibers. Clinically, 3 main groups of systemic findings involving skin, eye, and vessels emerge in the symptoms. In this report, we present the case of a patient with PXE who has agenesis of most of the permanent teeth. No previous reports in the English literature have mentioned oligodontia in PXE. Our treatment plan consisted of extraction of the upper left deciduous canine followed by fixed orthodontic treatment. Further prosthodontic rehabilitation was planned to restore the edentulous area.