Abstract
Nevoid basal cell carcinoma (BCC) syndrome is a rare syndrome caused by mutations in PTCH1 gene and characterized by several developmental anomalies and neoplasms affecting multiple organ systems. Conventionally, the syndrome was described as a triad of multiple BCCs, keratocystic odontogenic tumors (KCOTs), and bifid ribs. However, recently, we encountered a 25-year-old male patient who presented incidentally with multiple KCOTs, bifid ribs, and other characteristic findings, but not BCCs. This case along with appropriate literature review is being presented herewith.
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