Basal cell carcinoma (BCC) is the most common skin cancer. Metastatic BCC is an extraordinary rare finding observed in only 0.5% of all cases. Until the introduction of the small molecule hedgehog inhibitor vismodegib, patients with metastatic BCC were treated with chemotherapy, most frequently platinum-based with mixed responses to therapy. We present the case of a 55-year-old Caucasian man who suffered from BCC on his left arm with lymph node and pulmonary metastases. Sonic hedgehog blockade with vismodegib only induced a short remission, and the patient succumbed to the cancer.