Basal cell carcinoma (BCC) is the most common non-melanocytic skin cancer. Therapeutic strategies available so far include: surgical resection, radiotherapy, or — in the case of low risk of recurrence — superficial application of drugs like 5-fluorouracil or imiquimod. The frequent localisation of BCC is eye bulb area, often with deep penetration into the tissues. Standard therapies in this area may lead to blindness. The Hedgehog (HH) pathway is a key point in BCC development. For patients with advanced BCC, not suitable for surgical resection or radiotherapy, oral small-molecule drug inhibitors of this pathway are the new therapeutic option. Here we present a case of a patient with locally advanced skin BCC localised on the face, sinuses, and eye-socket region. Wide excision with the right eye bulb resection was performed. Eight months after the treatment the patient developed regional recurrence. Due to the advanced stage of the disease the patient was disqualified from further surgical resection. Use of radiotherapy was associated with high risk of blindness of the left eye. The patient was treated with vismodegib and developed a complete response. Moreover, we avoided blindness after radiotherapy. Also, the patient’s symptoms due to locally spreading cancer resolved. The patient has been receiving HH inhibitor without any progression in imaging tests for 20 months