Aim: We present the management of a severe case of recurrent periocular basal cell carcinoma, orbital invasion and exenteration. Case report: The present case is of a recurrent basal cell carcinoma in a 84-year-old male presenting with non-healing lesion above right eyelid. A tumor excision was performed in May 2014. Histopathology revealed a basal cell carcinoma (dg. C44.1 ICD-10-CM) with positive margins. The re-excision of the lesion was performed. After two years, there was a local recurrence and orbital invasion. Indication for external curative radiation therapy. Plastic surgery of the upper eyelid. Orbital exenteration was indicated in January 2018. After another year, a recurrence of the tumor was once again noted. Histopathology revealed a basal cell carcinoma (dg. C44.1 (TNM 7, pMx, pNx, pTx)). The patient was indicated for external radiotherapy. There were no indications for biological treatment. After another year, a progression of the local finding was noted. Conclusion: Basal cell carcinoma (BCC) is the most common non-melanoma skin cancer of the periocular region. Primary treatment of basal cell carcinoma is surgical. Advanced lesions require extensive surgical interventions and other available treatment modalities. In some cases, mutilating surgery – exenteration of the orbit is inevitable. Despite a relatively small percentage of invasive diseases today, advanced stages may still occur; either as a result of the patient's late presentation, inadequate initial therapy aimed at maintaining critical periorbital structures, or due to high tumor aggression. The case report highlights necessity of radical resection of primary tumor with histological examination.