Background:  Platelet‐derived growth factor (PDGF)‐AA isoform and its receptor, PDGF‐α receptor (PDGFRA) regulate tooth development and growth. We investigated the expression of both proteins in ameloblastomas, to contribute the understanding of the potential role of the PDGF/PDGFR system in this odontogenic neoplasm.Method:  Twenty‐nine specimens of ameloblastoma were analyzed for PDGF‐AA and PDGFRA expression using immunohistochemistry. The proliferation activity was investigated with the MIB‐1 antibody. Additionally, capillary sequencing of genomic DNA was performed to search for mutations in therapeutically relevant exons 12 and 18 of the PDGFRA gene.Results:  PDGF‐AA and PDGFRA expression were detectable in all cases with the exception of one tumor. However, protein expression levels did neither correlate with each other nor with MIB‐1 expression. Unicystic ameloblastomas did not differ from solid tumors with regard to PDGF‐AA, PDGFRA, and MIB‐1 expression. One tumor revealed a somatic mutation of exon 12 of the PDGFRA gene.Conclusion:  PDGF‐AA and PDGFRA proteins are regularly expressed in variable levels in ameloblastomas, and somatic mutations of exon 12 and exon 18 of the PDGFRA gene are rare findings.