Odontogenic keratocysts (OKCs) are benign yet locally aggressive cystic lesions derived from the dental lamina or its remnants. Predominantly found in the mandible, OKCs often present as asymptomatic radiolucent lesions, occasionally causing symptoms such as swelling and pain. Radiographically, they appear as well-defined, unilocular or multilocular lesions with smooth borders. Microscopically, OKCs feature a thin, parakeratinized epithelium with a high mitotic index, contributing to their aggressive behavior. Treatment typically involves surgical enucleation or resection, with careful attention to ensuring complete removal to minimize recurrence. However, recurrence rates remain relatively high, ranging from 25% to 60%, necessitating close postoperative monitoring, particularly in cases associated with nevoid basal cell carcinoma syndrome. A multidisciplinary approach involving oral and maxillofacial surgeons, pathologists, and geneticists may be required, especially in cases of syndromic association. Early detection and management of recurrences are essential for optimal patient outcomes.