Colorectal carcinoma (CRC) is extremely rare among pediatrics, particularly before puberty. It usually begins with nonspecific signs and symptoms, so the index of suspicion is low, consequently, the diagnosis may be delayed. Reports in the literature indicate that poor prognostic factors are more common in children than in adults, resulting in a worse outcome. Our objective is to report clinical profile, treatment and prognosis of CRC in children and adolescents admitted in our institution, and to perform a systematic review of reports in the bibliography.