Italy has one of the highest paediatric cancer incidence rates in Europe. We compared cancer incidence and survival rates in children (0-14 years) and adolescents (15-19 years) residing in Palermo Province (PP) with statistics derived from Italian and European surveillance systems. We included all incident cancer cases, malignant tumours and non-malignant neoplasm of central nervous system (benign and uncertain whether malignant or benign), detected in children and adolescents by the Palermo Province Cancer Registry (PPCR) between 2003 and 2012. A jointpoint regression model was applied. Annual Average Percentage Changes were calculated. The Besag-York-Mollie model was used to detect any cluster. The 5-year survival analysis was computed using Kaplan-Meier and actuarial methods. We identified 555 paediatric cancer incident cases (90% "malignant tumours"). No difference in incidence rates was highlighted between PPCR and Italy 26 registries and between PPCR and Southern Europe. No jointpoint or significant trend was identified and no cluster was detected. The 5-year overall survival didn't differ between PP and the Italian AIRTUM pool. A borderline higher statistically significant survival was observed in age-group 1-4 when comparing PPCR to EUROCARE-5. The epidemiological surveillance documented in the PP was a paediatric cancer burden in line with Italy and southern Europe. The study supports the supplementary role of general population-based cancer registries to provide paediatric cancer surveillance of local communities.