Abstract
A 63‑year‑old man with a history of heavy tobacco use presented to the hospital with a 2 × 3 cm necrotic lesion of the left temporal region associated with pain and progressive vision loss in the left eye. Biopsy of the left lateral orbital wall demonstrated locally invasive basal cell carcinoma (BCC). The patient was referred to a National Cancer Institute-designated comprehensive cancer center for further management. He was initiated on neoadjuvant hedgehog pathway inhibition with vismodegib and had an excellent oncologic response. Despite this, visual acuity in the left eye continued to decline and was accompanied by worsening proptosis and pain, which was refractory to multimodal analgesia. The patient was referred to ocular oncology, and vismodegib was discontinued after six months of treatment in anticipation of surgery. The patient underwent left eye enucleation and reported complete resolution of pain. He showed no evidence of residual disease four months after completion of vismodegib therapy. This report highlights a rare case of locally advanced periocular BCC with orbital invasion managed with neoadjuvant hedgehog pathway inhibition and palliative enucleation.