OBJECTIVES: Limited data regarding intracranial mesenchymal chondrosarcoma (MCS) are available. The goal of this study was to report the clinical characteristics, challenges in management, and poor outcomes of intracranial MCS. METHODS: Clinical data for 16 patients with MCS were reviewed retrospectively to evaluate their clinical characteristics, management, and outcomes. RESULTS: This study included 11 male and 5 female patients with a mean age of 22.9 +/- 14.4 years. The most common presentations were headache (n = 10; 62.5%), followed by cranial deficits (n = 7; 43.6%). The radiologic spectrum for MCS was broad, and only 18.8% (3/16) of MCSs were correctly diagnosed preoperatively. Aggressive resection (including subtotal resection and gross total resection) and partial resection was performed in 62.5% (10/16) and 37.50% (6/16) of patients. With a median follow-up of 34 months (range, 10-78 months), 5 patients (31.3%) died and 8 patients (50%) developed tumor recurrence. The 1-, 3-, and 5-year rates of progression-free survival and overall survival were 86%, 53%, and 42% and 93%, 70%, and 56%, respectively. Although the differences were not significantly different, aggressive resection and the use of radiotherapy tended to improve the prognosis of the patients. CONCLUSIONS: Clinical characteristics of MCS are variable. The current management of intracranial MCS referring to conventional chondrosarcoma could not yield satisfactory outcomes. Further study is needed to identify the optimal treatments.