Mutations in the WNT10A gene have been described in three ectodermal dysplasia conditions, odontoonychodermal dysplasia (OODD; MIM 257980), Schopf-Schulz-Passarge syndrome (SSPS; MIM 224750) and tooth agenesis, selective, 4 (STHAG4; MIM 150400). OODD and SSPS are autosomal recessive disorders with overlapping clinical findings. The findings include dry hair, severe hypodontia, smooth tongue with marked reduction of fungiform and filiform papillae, onychodysplasia, keratoderma, and hyperhidrosis. Additional findings in SSPS include cysts of the eyelids and an increased risk of benign and malignant skin tumors. STHAG4 can be caused by homozygous, heterozygous or compound heterozygous mutations in WNT10A. It is characterized by isolated oligodontia. Typical findings include absent premolars and molars, and missing or peg-shaped maxillary incisors. Isolated hypodontia of maxillary permanent canines have also been reported.