Florid cemento-osseous dysplasia (FLCOD) is a rare, extensive bone metabolism disorder, which occurs only in the jaw bone. It is usually asymptomatic for a long time and discovered incidentally during a radiological examination. The characteristics of FLCOD in the initial stages are similar to those of periapical granuloma or jaw cyst, which may lead to misdiagnosis. After the lesion is mature, the imaging findings show that radiopaque with a thin radiolucent peripheral halo, which is crucial for the diagnosis of FLCOD, but other jaw lesions have similar imaging findings. Due to the poor blood supply of the lesion, the alveolar bone of root apices of vital teeth is slow to heal after trauma, increasing the chance of infection, which can lead to the osteomyelitis of the jaws and emerge sequestrum. This paper reviews the aspects of pathogenesis, clinical characteristics, diagnosis, differential diagnosis and treatment.