Summary

Gorlin-Goltz syndrome is a rare genetic disorder with autosomal dominant inheritance, characterized by an increased predisposition to develop multiple basal cell carcinomas, odontogenic cysts, and skeletal abnormalities. Diagnosis is based on specific clinical and genetic criteria, considering the phenotypic variability and progressive evolution of the disease.

We present the case of a 50-year-old patient diagnosed with Gorlin-Goltz syndrome, who developed multiple pigmented, ulcerated basal cell carcinomas predominantly located on the trunk and cephalic extremities, with advanced locoregional invasion.

The prognosis of these patients is reserved due to the high risk of recurrence and local invasion, as well as significant aesthetic and functional complications. The disease also has a major psychological and social impact, determined by the need for repeated interventions and impaired quality of life. This case highlights the importance of early diagnosis and a multidisciplinary approach.