Basal cell carcinoma (BCC) is the most common skin malignancy. For locally advanced or metastatic cases not amenable to surgery or radiotherapy, Hedgehog pathway inhibitors represent an effective treatment option. A 50-year-old female, previously treated with surgery, radiotherapy, and chemotherapy, presented with recurrent BCC invading the mastoid bone and dura. The tumor was deemed unresectable, and neoadjuvant carboplatin-docetaxel showed minimal benefit. Vismodegib 150 mg daily was initiated, resulting in significant clinical and radiological regression. Sustained disease control has been achieved for five years without major toxicity. This case highlights the durable efficacy of vismodegib in aggressive, locally advanced BCC and supports its use as a valuable therapeutic strategy when conventional modalities are not feasible.