Enchondromas (EC) are frequent incidental findings on magnetic resonance imaging (MRI) performed for the diagnosis of joint pathology, especially observed on MRI examinations of the knee and shoulder. Enchondroma has potential for malignant transformation to chondrosarcoma (CS), and it may be difficult to distinguish EC from low-malignant CS on the basis of imaging or histopathology studies. Therefore, EC is mostly followed up to monitor any growth and/or changes indicating aggressive tumor.There is no consensual evidence on when and how to follow up patients with EC with regard to potential malignant transformation. Therefore, the Danish Multidisciplinary Cancer Groups initiated and supported the elaboration of Danish guidelines in 2020 based on a literature review. The guidelines are presented here, in addition to a summary of the background literature.