Pediatric primary central nervous system (CNS) melanoma is a rare and aggressive malignancy, often arising in association with neurocutaneous melanosis but also reported in sporadic cases. Children typically present with signs of increased intracranial pressure, seizures, or focal neurological deficits. Median age at diagnosis is 2.8–3.7 years, that is, under four years, although cases spanning infancy to early adolescence have been reported. Median survival rarely exceeds one year despite multimodal therapy. Melanogenesis may directly influence tumor behavior, as intermediates generate oxidative stress and contribute to radioresistance and variable immunotherapy response. Pediatric CNS melanomas frequently harbor activating mutations in GNAQ, GNA11, or NRAS, which drive mitogen-activated protein kinase and phosphoinositide 3-kinase–AKT signaling to promote proliferation and confer resistance to apoptosis. Additional alterations, such as CDKN2A and TP53 loss, may be linked to accelerated progression. Unlike cutaneous melanoma, BRAF mutations are uncommon. Maximal surgical resection remains the cornerstone of therapy, offering the greatest survival benefit. Adjuvant radiotherapy or chemoradiation provides limited efficacy due to the intrinsic radioresistance of melanoma cells and the restricted delivery of therapeutic agents across the blood–brain barrier (BBB). Among chemotherapies, temozolomide has modest CNS activity, whereas checkpoint inhibitors and targeted agents have shown only transient responses in pediatric case reports. These therapeutic challenges underscore the urgent need for novel strategies that can overcome BBB-related barriers to drug delivery and melanin-mediated resistance. Advances in molecular profiling and targeted drug development, coupled with education for early diagnosis, may improve survival in patients with this aggressive disease.