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Gorlin-Goltz syndrome, also known as basal cell nevomatosis (CBN), is a rare inherited disorder belonging to the family of neurocristopathies or diseases caused by abnormalities of the neural ridges. We report the case of 4 patients, suffering from this syndrome, followed up in the stomatology and maxillofacial surgery service in Casablanca, including a familial form in two cases: a mother and her son and 2 isolated cases, followed and treated in our service of maxillofacial surgery of the August 20 hospital in Casablanca. A clinical, biological and radiological assessment was made followed by surgical treatment for the 3 patients, an anatomo-pathological examination and the establishment of a strict monitoring plan.
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