Background Cooperative clinical trials has increased the knowledge on pediatric tumors, however, this is not the case for rare tumors (RT). Objective To describe the incidence, clinical characteristics and outcome of RT in the pediatric age diagnosed at Garrahan Hospital. Material and methods Retrospective descriptive study of patients (pts) between 0 and 18 years admitted between January 2 007 and December 2 017, with diagnosis of RT. Results Of 1 657 pts with diagnosis of solid tumors, 164pts (9.9%) corresponded to RT, 71.95% (118pts) were under 14 years old and 81.7% (130pts) were male. In order offrequency RT were: thyroid carcinoma (ca) 60pts, adrenal ca 14pts, lung tumors 14pts, melanoma 13pts, salivary glands ca 11pts, gastrointestinal tumors 8pts, non-gonadal germinal tumors 7pts, pancreatic tumors 7pts, renal ca 6pts, nasopharyngeal ca 5pts, pheochromocytoma/paraganglioma 5pts, timo 1pte. The treatment received depended on the type of tumor and stage. With a median follow-up of 34.9 months (range: 1- 128.5 months), 133pts (78.7%) are alive and only 10pts (6%) were lost to follow-up. Conclusion Knowing these initial data will allow us to propose new registration strategies and to develop multidisciplinary proposals for diagnosis, treatment and follow-up.