Background: Nevus Sebaceous (NS) is a rare congenital skin lesion affecting approximately 0.3% of all newborns. Whilst benign, NS can undergo malignant transformation. The published rate of malignant transformation varies. This meta-analysis aims to identify the rate of malignant and benign secondary neoplasms occurring in NS. Methods: A literature search was conducted using PubMed, Embase and Web of Science from inception until April 2023. Two independent reviewers screened studies, extracted data, and assessed the quality of included studies. The primary outcome was the pooled incidence of secondary neoplasms. Studies with sample sizes greater than 50 patients were eligible for meta-analysis using the random effects model. Results: Twenty-eight studies were identified, 22 of which were eligible for meta-analysis. The overall transformation rate of NS was 12.8% (95% Cl: 9.2% – 17.6%), malignant and benign transformation rate of NS was 2.4% (95% CI: 1.4% - 4.1%) and 10.3% (95% CI: 7.5% - 13.9%), respectively. The rate of NS transforming into BCC was 1.7% (95% CI: 0.9% – 3.2%) whereas the rate for the development of syringocystadenoma papilliferum was 3.6% (95% CI: 2.5% - 5.3%); trichoblastoma was 2.6% (95% CI: 1.7% - 3.8%). Conclusion: While the malignant transformation rate of NS is low, it is not negligible. Prophylactic early excision remains a viable approach to prevent secondary malignant neoplasms, address cosmetic and functional complications, and pre-empt the need for complex reconstruction in the future. We propose that resection of NS lesions in childhood remains a reasonable first line option in the appropriate patient keeping in mind that it may leave an undesirable scar.