To explore the clinical characteristics, differential diagnosis, surgical outcomes, and prognosis of primary intraspinal melanoma and pigmented neurilemmoma. Methods: A retrospective analysis was conducted on the clinical data of 5 patients with primary intraspinal melanoma and 7 patients with primary intraspinal pigmented neurilemmoma, all of whom underwent surgical treatment and were pathologically confirmed. The clinical characteristics, key points of differential diagnosis, surgical outcomes, and prognosis of these two diseases were analyzed. Results: The 12 patients exhibited non-specific clinical characteristics, primarily presenting with symptoms of spinal cord impairment at the corresponding lesion levels. On conventional MRI, both the 5 cases of primary intraspinal melanoma and the 7 cases of primary intraspinal pigmented neurilemmoma generally showed isointense or hyperintense signals on T1WI and isointense or hypointense signals on T2WI at the corresponding levels. There were certain differences in MRI contrast-enhanced imaging: primary intraspinal melanoma typically demonstrated heterogeneous enhancement with ill-defined borders and invasion of adjacent tissues, while primary intraspinal pigmented neurilemmoma mostly showed homogeneous enhancement with relatively well-defined borders. For the 5 cases of primary intraspinal melanoma, the surgical duration ranged from 80 to 212 minutes, with an average of (140 ± 46) minutes; intraoperative blood loss ranged from 100 to 600 mL, with an average of (260±100) mL; and there were no perioperative deaths. Postoperative MRI revealed complete resection in 2 cases, subtotal resection in 2 cases, and partial resection in 1 case. For the 7 cases of primary intraspinal pigmented neurilemmoma, the surgical duration ranged from 100 to 228 minutes, with an average of (150±48) minutes; intraoperative blood loss ranged from 120 to 580 mL, with an average of (250±80) mL; and there were no perioperative deaths. Postoperative MRI showed complete resection in all 7 cases. Among the 5 patients with primary intraspinal melanoma who were regularly followed up after surgery, 4 patients died within 3 to 15 months after discharge, with a median survival time of 8.5 months. The cause of death in all cases was distant metastasis. One patient had a survival time exceeding 24 months and showed no signs of local recurrence or metastasis as of the last follow-up (June 2024). All patients completed at least 6 months of follow-up. The 7 patients with primary intraspinal pigmented neurilemmoma underwent regular postoperative imaging monitoring. All patients were followed up for 12 to 60 months, and all were alive with no evidence of tumor recurrence or metastasis. Conclusion: Primary intraspinal melanoma and pigmented neurilemmoma exhibit non-specific clinical characteristics. MRI can be used for preliminary differential diagnosis, but pathological results are required for definitive identification. Primary intraspinal melanoma is more severe than pigmented neurilemmoma. Microscopic total tumor resection is the preferred treatment option, and postoperative adjuvant radiotherapy and chemotherapy can prolong patient survival. Pigmented neurilemmoma is mostly a benign tumor, and total tumor resection can generally be achieved. Postoperative radiotherapy and chemotherapy are usually not required, but long-term prognosis is difficult to predict.