Cemento-ossifying fibroma (COF) is classified as a fibro-osseous lesion and usually presents as a progressively growing lesion that can reach a large size if left untreated. Fibrous dysplasia (FD) is a non-neoplastic development that affects the craniofacial bones and is characterized by painless hypertrophy due to the replacement of bone by abnormal fibrous tissue. Herein, a case of concurrent occurrence of COF and FD involving the right mandible is described in a 14-year-old male patient. In this review, the current knowledge of FD and COF is summarized, emphasizing the importance of integrating the understanding of clinical, radiographic, and histopathological features. In addition, the relevant aspects associated with differential diagnosis and patient care are evaluated.