Background: Large Ceratocystis Odontogenic Tumor (LCOT) is a rare and aggressive odontogenic neoplasm that poses significant diagnostic and therapeutic challenges. This study aims to elucidate the molecular and genetic mechanisms driving LCOT pathogenesis to improve our understanding of this disease and its clinical management. Aim: The primary objective of this study is to investigate the underlying molecular and genetic alterations in LCOT, identify potential diagnostic markers, and explore therapeutic targets for personalized treatment strategies. Methods: Tissue samples from a cohort of LCOT patients were subjected to comprehensive molecular profiling using state-of-the-art techniques, including next-generation sequencing, gene expression analysis, and immunohistochemistry. Data analysis and bioinformatics tools were employed to uncover genetic mutations, altered signaling pathways, and potential biomarkers associated with LCOT. Results: Our study revealed a spectrum of genetic mutations and alterations in LCOT, highlighting the involvement of specific pathways, such as Wnt/β-catenin signaling, in tumorigenesis. Notably, we identified potential diagnostic markers and therapeutic targets that may pave the way for more precise diagnosis, improved prognosis assessment, and the development of targeted therapies for LCOT. Conclusion: The molecular and genetic characterization of LCOT presented in this study provides valuable insights into the complex pathogenesis of this rare tumor. By identifying diagnostic markers and potential therapeutic targets, this research opens new avenues for the development of personalized treatment strategies, ultimately improving the clinical management and outcomes for LCOT patients.