Basal cell carcinoma is the most common malignancy of humans. Although it is axiomatic that this tumor does not evolve into metastatic disease, such events rarely occur, and this possibility should not be overlooked.The reader should better understand the sequence of events that resulted in metastatic disease and how these events are emblematic of the rare cases of basal cell carcinoma that systemically spread.We present a case report of basal cell carcinoma that underwent distant metastasis. A short review of the literature is included.Although basal cell carcinoma is commonly considered a regional tumor with virtually no propensity for distant spread, this case reveals that metastatic disease does occur and with devastating results.Metastatic disease in basal cell carcinoma is a very rare but catastrophic consequence of this very common skin malignancy.