Introduction: Tumors of the central nervous system (CNS) in children between 0 and 2 years of age are infrequent, so their diagnosis and treatment constitute an important challenge for every pediatric neurosurgical center, to which is added the comorbidities typical of this age that condition the responsibility of improving therapeutics to obtain better survival. The objective of this study was to describe the diagnostic and therapeutic experience of a neurosurgical Department of national reference, in relation to neoplasms of the central nervous system in children under two years of age, as well as to establish comorbidity and prognosis.

Methods: Cross-sectional, retrospective and descriptive study that includes all patients under 2 years of age who were diagnosed with a neoplastic lesion of the central nervous system, attended from January 01, 2016 to July 01, 2019 at the Baca Ortiz Pediatric Hospital from the city of Quito in Ecuador.

Results: CNS tumors in children between 0 and 2 years old corresponded to 5.09%, with irritability being the most frequent reason for consultation with 62.5%. Also, 75% of the neoplasms were located at supratentorial level with a 1: 1 intra / extra-axial ratio. Neuroblastoma and choroid plexus tumors were the most frequent histopathological diagnoses. Mortality had a percentage of 50%.

Conclusions: CNS tumors in children between 0 and 2 years are not frequent, the location is predominantly supratentorial and the prognosis for life depends on the histopathological type. Radiation therapy is an option, although surgery for resection is the basis of treatment.