Childhood melanoma (ChM) is rare, with clinical and epidemiologic characteristics that differ from those of adult melanomas. The objective of the current study was to systematically identify and analyze case reports and case series of fatal and metastasizing ChM in the medical literature. ChM case reports with a fatal outcome or metastases were identified using a Medline search and subdivided into ChM developing in the absence of a congenital melanocytic nevus (ChM without CMN) and ChM associated with a CMN (ChM with CMN); 258 cases of ChM without CMN (206 cutaneous, 52 noncutaneous) were identified. In cutaneous ChM without CMN with a fatal outcome (n= 155), the mean age at diagnosis was 13.1 years (median 14 yrs). The mean Breslow index in this group was 8.5 mm for children ages 0 to 10 years and 3.7 mm for children ages 11 to 18 years. In ChM with CMN (n= 178; 112 cutaneous, 66 central nervous system [CNS]), the mean age at diagnosis was 5.8 years for cutaneous melanoma (median 3 yrs) and 5.5 years for CMN-associated CNS melanoma (median 3 yrs). The majority of CMN-associated cutaneous melanomas developed in small and giant CMN (vs medium and large); 53.9% of CNS melanomas developed in patients with multiple medium CMN. This study represents the largest and most complete synopsis of ChM case reports in the medical literature. Our analysis supports the view that cutaneous ChM without CMN (or associated with smaller CMN) differs in several important aspects from ChM associated with large or giant CMN.