Cutaneous neoplasms are relatively rare in children. Most commonly, skin cancers arise through environmental factors, particularly ultraviolet radiation
thus, age is the most predictive factor in developing cutaneous carcinomas. However, children born with certain genodermatoses are significantly more likely to develop malignancies and must be carefully monitored and treated. Most published data are based mainly on signs and symptoms present in White patients. Therefore, we aim to highlight the cutaneous presentations and relative differences of these genodermatoses among patients with skin of colour, who are underrepresented in medicine. We conducted a literature review of 504 patients presented in 236 published articles. Manuscripts with accessible case reports for children aged ≤ 17 years were included. Patients with skin of colour often present with fewer classical findings and have higher incidences of scarring and dyspigmentation. There is also a higher incidence of consanguinity in affected patients. Providers who are able to recognize nonclassical signs can provide proper management and treatment regimens, potentially bringing outcomes for patients with skin of colour more in line with those of White children.