Pleomorphic adenoma affecting the lachrymal gland requires a well-grounded clinical and therapeutic protocol to avoid the risk of malignant transformation or disease recurrence, which is quite dangerous at this site. The authors present a clinical case of pleomorphic adenoma recurrence affecting the right lachrymal gland in a patient who first underwent a biopsy, with subsequent resection of the neoplasm. The authors also review the clinical and radiological features enabling a differential diagnosis and describe the radical “exenteratio orbitae” surgery for pleomorphic adenoma recurrence 7 years after the first operation, which was performed because of total derangement of the orbital cavity. The patient remains disease-free 5 years after this surgical treatment. Therefore, it turns out that an incisional biopsy carries a higher biological cost for the patient.