The Calcifying Odontogenic Cyst (COC) is a simple cyst lined by ameloblastoma-like epithelium with ghost cells. The peripheral COC is a rare lesion and few reports have been published considering its clinical and histopathological features. This article aimed to report on a case of a peripheral COC, discussing its clinical, imaginological and histopathological features. A 9-year-old male patient presented a 10x5 mm painless nodule in the palatal mucosa of the left central incisor. Panoramic, occlusal and periapical radiographs did not show alterations. A computed tomography exam showed a slight soft tissue swelling located in the palatal mucosa of the left maxillary central incisor. An excisional biopsy was performed. The histopathological analysis showed a cystic lesion adhered to an oral mucosa fragment and lined by an ameloblastoma-like epithelium with ghost cells. The diagnosis of peripheral COC was established and the patient has been disease-free for 5 years. Although rare, peripheral COC is an important lesion that should be considered as a differential diagnosis of gingival hyperplastic lesions.