AbstractPaediatric melanoma, although rare, is the most common skin cancer in children. Our current knowledge on paediatric melanoma incidence trends is expanding, as several studies have addressed this issue with conflicting results. Known risk factors for paediatric melanoma include family history of melanoma, a previous history of malignancy, large congenital nevi, numerous melanocytic nevi, sunburns, increased UV exposure and a sun‐sensitive 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 ABCDE criteria. The major differential diagnoses are melanocytic nevi, proliferative nodules in congenital nevi and atypical Spitz tumours. Moreover, in the younger age group non‐Caucasian children are over‐represented, 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. Specific guidelines for management of melanoma in children do not exist, and most often the disease is treated similarly to melanoma in adults.