Gorlin’s Syndrome (basal cell nevus syndrome) is a rare autosomal dominant disease which characterized with multiple basal cell carcinomas (BCC), maxillamandibular odontogenic keratocysts, palmoplantar pits and various skeletal anomalies. Despite its frequency, BCC has a low mortality rate because of its slow progression and minimal soft tissue invasion, however, it can occasionally show aggressive course with deep invasions, metastasis and recurrences. In this article we reported a Gorlin’s syndrome case which presents with BCC , showing perineural invasion and relapsing consistently after surgical treatments. Our aim is to remind the hereditary diseases in the early onset cases with multiple BCC and to draw attention to aggressive course of BCC which located especially in periorbital and perinasal areas.
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