The discovery of increased mitotic activity in the cyst epithelium, the potential for epithelial budding from basal layer or daughter cysts in the cyst wall, the presence of chromosomal abnormalities, and the role of mutation of the PTCH gene in the etiology of KCOT resulted in its reclassification and renaming as keratocystic odontogenic tumor [1–6]. The gene discussed is PTCH1; the disease is Keratocystic odontogenic tumor.