BACKGROUND : Basal cell carcinomas are the most common periocular malignant tumor. In advanced periocular basal cell carcinoma, vismodegib is a new treatment option which might potentially avoid surgical eye removal. METHODS : We treated a 76-year-old patient unwilling to consent to surgery with vismodegib for advanced periocular basal cell carcinoma on the left forehead that had already undergone several previous treatments. After initial partial remission, the tumor regrew under ongoing therapy, so that radical surgical excision including orbital exenteration was performed. Unfortunately, the patient died thereafter due to septic multi-organ failure. CONCLUSIONS : Basal cell carcinoma and its new treatment options are gaining importance for ophthalmology due to rising incidence and prevalence rates. Vismodegib is a new encouraging option. However, for advanced tumors, it must be resolved whether complete histological remission may be achieved to avoid surgical intervention, or whether the area of resection can be significantly reduced. Current multicenter studies investigate these aspects further (ClinicalTrails.gov identifier: NCT03035188).