Introduction: Maffucci syndrome is characterized by multiple subcutaneous enchondromas and can be complicated by skull base chondrosarcoma, a rare malignant tumor that produces a cartilaginous matrix that invades the cortical bone and medullary tissue. Objective: To review this syndrome and its correlation with chondrosarcoma. Method: Narrative review based on published information with material for reading and analysis selected from virtual publishers, SciELO, VHL - Virtual Health Library, Pubmed and Scopus. Initially, a search was carried out for descriptors related to the topic, existing in DeCS/MESH using the following terms: “skull base tumor, chondrosarcoma, Maffucci syndrome” with AND or OR search, considering the title and/or summary. Result: After analyzing the relevant articles, 40 articles were selected and included in this review. Conclusion: Skull base lesions should be highly suspected in patients with Maffucci syndrome.