Background: Vismodegib used in the treatment of metastatic basal cell carcinoma (BCC) or locally advanced, recurrent BCC not amenable to surgery or radiation leads to various clinical changes. Objective: Aim was to elucidate the histopathology that corresponds to tumor involution observed with vismodegib therapy. Methods: Retrospective case series of patients treated with vismodegib between May 2012 and April 2017 with intra- or post-treatment biopsy. Results: 42 biopsy specimens and 4 Mohs frozen sections were analyzed. Necrosis, fibrosis, and increased plasma cells were common features. Limitations: Single center study. Conclusion: The histologic findings of BCCs treated with vismodegib correlate with clinical response. J Drugs Dermatol. 2019;18(2):136-138.