CCOC mainly occurs in the 6th decade of life and predominantly affects women, in contrast to the case of a young man reported here [1, 5]. Consistent with our study, Labrador et al. observed that the mandible (82%) was concerned significantly more often than the maxilla (18%) [1]. In the present case, the patient’s only symptom was a painless swelling of the left mandible. Swelling or lumps (80%) were also the most common clinical symptoms in the literature, while pain occurred in only 41% of the 117 patients described. [1] Due to the nonspecific clinical and radiological features of CCOC, immunohistology is of particular importance. As initially, both benign (e.g., cystic lesions) and malignant processes (e.g., squamous cell carcinoma) are possible differential diagnoses. Before histopathological confirmation, a whole series of clear cell containing tumors must be considered as a differential diagnosis. Other odontogenic tumors (amyloid-rich odontogenic fibroma, calcifying epithelial odontogenic tumor, or odontogenic carcinoma with dentinoid), salivary gland tumors (clear cell carcinoma, mucoepidermoid carcinoma, or epithelial myoepithelial carcinoma), or distant metastases (renal clear cell carcinoma or malignant melanoma) must be ruled out. [6] In line with Ebert et al., therapeutic treatment of CCOC requires primary surgical tumor resection with adequate safety margins [5]. Depending on tumor staging, the necessity and extent of cervical lymph node dissection and adjuvant radiotherapy are determined. Following a one- or two-stage approach, reconstruction of bone and soft tissues completes the comprehensive interventional procedure. Continuous follow-up is crucial for the patient’s long-term outcome.