An 18-year-old male patient reported with a chief complaint of a recurrent swelling and dull aching pain in upper left back region of the jaw since 1 mo. Patient was apparently alright 1 mo back until he experienced dull aching pain in upper left posterior region of the jaw. Patient was otherwise healthy 4 years back until he noticed a swelling in upper left region of jaw which slowly increased to a large size. He visited a hospital at his native place and was operated twice one year apart for the same swelling. The swelling reduced in size but did not disappear completely. So, the patient reported to the Department of Oral Pathology and Microbiology with the chief complaint of recurrent swelling and dull pain in upper left region of the jaw. The past medical history of the patient was not contributory. Not contributory. Extraoral examination revealed a diffuse swelling of approximately 7 cm × 5 cm in size on the left side of face extending antero-posteriorly from left ala of nose to the anterior border of the ramus and supero-inferiorly from infraorbital rim to the corner of the mouth. Skin over the swelling was normal. On palpation, the swelling was found to be bony hard in consistency. Temperature over the swelling was slightly raised. A single, left submandibular lymph node was palpable approximately 2 cm × 2 cm in size. Intraorally, a single, smooth, ovoid swelling, extending antero-posteriorly from distal of 22 to mesial of 26 and supero-inferiorly from vestibular depth to marginal gingival was noticed. On palpation, it was bony hard and slightly tender with fixity to underlying bone. Computed tomography (CT) scan showed a mixed hypodense hyperdense lesion in maxillary left region extending antero-posteriorly from the distal aspect of 21 up to 26 regions and supero-inferiorly from alveolar ridge upto the floor of orbit. The lesion had a well defined, partly corticated periphery. It had predominantly hypodense internal structure with multiple intermittent hyperdense flecks present within. Expansion was evident on the buccal aspects of the alveolus, anterior and lateral walls of the left maxillary sinus with thinning and perforation evident at multiple sites. Thinning of the floor of left orbit with invagination of the lesion was noticed. Based on the clinical and radiographic findings, provisional diagnosis of benign odontogenic tumor was arrived at. Due to the extent of the lesion and history of recurrence, CCOT was considered. Following routine blood investigations, patient was referred to the department of oral surgery for incisional biopsy of the lesion. Careful histopathological examination of Hematoxylin and Eosin (H and E) stained sections showed the following features: cystic lumen was lined by odontogenic epithelium of variable thickness. Basal cells of the epithelium were tall columnar with polarized hyperchromatic nuclei. Stellate reticulum like cells could be noted above the basal cells. The superficial layers showed groups of pale eosinophilic ghost cells. The connective tissue wall was predominantly fibrous with dense bundles of collagen fibres and devoid of inflammation. Many active odontogenic rests were also seen in the connective tissue. One or two areas demonstrated globular areas of calcifications. The histopathological diagnosis of the incised biopsy specimen was given as COC. Segmental resection was carried out in this case and the specimen was subjected to H and E staining. The histopathological examination revealed a connective tissue wall with odontogenic epithelium. At few places, the epithelium was proliferating with stellate reticulum like cells surrounded by spindle shaped cells. Aggregates of eosinophilic ghost cells surrounded by irregular calcifications could be noted towards the lumen. Large areas of dentinoid were conspicuously present in the subjacent connective tissue. At places, active odontogenic rests and metastatic bone was seen. Special staining with van Gieson’s stain identified dentinoid which stains pinkish red and ghost cells which appear yellow in colour.