Osteopoikilosis (OPK) is a rare, benign, and asymptomatic bone dysplasia that is developed during childhood and persists throughout life. This condition is generally found incidentally on plain radiographies made by other reasons. The main differential diagnosis is osteoblastic metastasis. So, OPK must be in differential diagnosis when bone lesions are identified on plain radiograph to avoid alarming the patient with more serious disease and misdiagnosis. In this paper, we review the clinical manifestation, pathophysiology, diagnosis, and treatment of OPK.