Highly prevalent activating EGFR mutations were identified in the vast majority of IP cases and also are commonly present in ISP-associated sinonasal squamous cell carcinoma but not in ESP, OSP, or sinonasal squamous cell carcinoma without a known papilloma association.[20] Some studies have indicated that KRAS mutations may be a disease-defining molecular feature of sinonasal oncocytic tumors.[21] In this case report, KRAS mutations were found in the OSP-associated adenocarcinoma. This evidence concerns the gene KRAS and incontinentia pigmenti.