Introduction. Odontogenic keratocyst (OKC) is a rare developmental, epithelial and benign cyst of the jaws of odontogenic origin with high recurrence rates. The third molar region, especially the angle of the mandible and the ascending ramus are involved far more frequently than the maxilla. The choice of treatment approach was based on the size of the cyst, recurrence status, and radiographic evidence of cortical perforation. Different surgical treatment options like marsupialization, decompression, enucleation, enucleation with Carnoy’s solution, peripheral ostectomy with or without Carnoy’s solution, and jaw resection have been discussed in the literature with variable rates of recurrence. Case report. We presented a 52-yearold male with orthokeratinized odontogenic keratocyst. Elliptical unilocular radiolucency located in the third molar region and the ascending ramus of the mandible, 40 × 25 mm in diameter with radiographic evidence of cortical perforation at the anterior ramus border of the mandible 20 mm in diameter, was registrated on orthopantomographic radiography. Surgical treatment included enucleation of the cyst and peripheral ostectomy with the use of Carnoy’s solution and excision of the overlying attached mucosa. Postoperatively, no paresthesia in the inervation area of the inferior alveolaris nerve was registrated. Recurrences were not registrated within 5 years post-intervention. Coclusion. Treatment of odontogenic keratocyst with enucleation and peripheral ostectomy with the use of Carnoy’s solution and excision of the overlying attached mucosa had a very low rate of recurrence. Radical and more aggressive surgical treatments as jaw resection should be reserved for multiple recurrent cysts and when OKC is associated with nevoid basal cell carcinoma syndrome (NBCCS). Following the treatment protocol in the management of OKC and systematic and long-term postsurgical follow-up are considered key elements for successful results.