Introduction Colorectal carcinoma (CRC) is one of the most common tumors in adult, but is extremely rare in children. In childhood, CRC often presents unfavorable aggressive histotypes, advanced clinical stage at onset and a worse prognosis. Pediatric CRC series are limited and include few patients, therefore information about treatment strategy and pharmacotherapy is scarce. For this reason, management of these patients represents a real challenge for pediatric oncologists.Areas covered The authors provide an overview of the general features and management strategies of pediatric CRC with specific attention to systemic treatment. Literature data regarding pharmacotherapy in published pediatric series are summarized and analyzed in detail, according to adult treatment standards.Expert opinion In the absence of specific recommendations for pediatric CRC, the general therapeutic strategy should follow the same principles as for adults and should be the result of a multidisciplinary discussion. Patient access to optimal treatment is difficult due to the lack of new drugs approved for the pediatric age group and non-availability of clinical trials. Collaboration between pediatric and adult oncologists is considered crucial in order to overcome these issues and find solutions to increase knowledge and improve the outcome of such a rare disease in children.