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Known risk factors for paediatric melanoma include family history of melanoma, a previous history of malignancy, large congenital nevi, numerous melanocytic nevi, sunburns, increased <jats:styled-content style=\"fixed-case\">UV<\/jats:styled-content> exposure and a sun\u2010sensitive phenotype. In younger children, melanoma more often presents with atypical features, such as a changing, amelanotic or uniformly coloured, often bleeding lesion, not fulfilling in most cases the conventional <jats:styled-content style=\"fixed-case\">ABCDE<\/jats:styled-content> criteria. The major differential diagnoses are melanocytic nevi, proliferative nodules in congenital nevi and atypical Spitz tumours. Moreover, in the younger age group non\u2010Caucasian children are over\u2010represented, tumours tend to be thicker and lymph nodes are often involved. Despite the frequent diagnosis at an advanced stage, the overall survival is fair in paediatric melanoma. 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