Introduction: Osteosarcoma and chondrosarcoma are malignant mesenchymal neoplasms that form bone and cartilage, respectively. Both neoplasms do not have pathognomonic signs, with the chondroblastic variety of osteosarcoma being similar to chondrosarcoma, so differentiating them is a challenge. The purpose of the present review was to characterize the epidemiology, imaging, and histology of craniofacial chondro blastic osteosarcoma (OSCCF) and craniofacial chondrosarcoma (CSCF) for similarities and differences. Materials and methods: A systematic review of clinical cases and clinical case series of OSCCF and CSCF was performed. Literature from the last 10 years in PubMed and Scopus was included. Epidemiological, imaging and histological characteristics were analyzed and compared between both neoplasms. Results: 35 cases were included, 15 corresponding to OSCCF and 20 to CSCF. Differences were found in location, time of evolution, bone expansion, bone perforation and degree of malignancy. No differences were found in age, sex, signs and/or symptoms, radiodensity, imaging pattern, signal intensity, limits, cortical effacement and/or infiltration, periosteal reaction, periodontal space, tooth displacement, external rooth resorption, advancement to adjacent anatomical regions, lymph node involvement, metastasis and immunohistochemical analysis. Conclusion: OSCCF and CSCF had no epidemiological, imaging or histological characteristics that differentiate them. Most of the characteristics analyzed presented considerable similarities.