Résumé : ABSTRACTIOsteopoikilosis (OPK) is a rare autosomal dominant hereditary disease with a prevalence estimated at 1/50000. Most of the time asymptomatic and benign, it can be misdiagnosed as other osteoblastic bone diseases (such as bone metastases) and lead to unnecessary stress and costly investigation. We report a 15-year-old boy with a complicated knee injury and associated OPK as well as a literature review.KEYWORDS Osteopoikilosis, Multiple sclerotic lesions, sclerosing bone dysplasia