Ollier disease (OD) is a subtype of enchondromatosis. Historically, it has been distinguished from Maffucci syndrome (MS) by the presence of vascular malformations and nonskeletal neoplasms (NSN) in the latter. However, there is an increasing number of reports of NSN in OD, and this categorization is now being questioned. We report a case of OD complicated by multifocal astrocytoma in a young patient, once again pointing to a possible association between OD and NSN. We also review the available literature and examine the similarities between the reported cases.