Introduction: Atypical teratoid rhabdoid tumors are highly malignant neoplasms that present in young children and can grow to a large size. Maximal safe surgical resection is a mainstay of treatment. Presentation of cases: Two cases of children under the age of two with large tumors involving the supratentorial and infratentorial compartments are presented. A two-staged operative approach combining a standard suboccipital approach to the fourth ventricle followed by an infratentorial, supracerebellar approach was utilized for resection. Discussion: Maximal safe surgical resection of large tumors in young children is challenging. A staged approach is presented that affords maximal tumor resection while minimizing perioperative morbidity. Conclusion: A staged operative approach appears safe and efficacious when resecting large tumors from both the infratentorial and supratentorial compartments in children less than two years of age.
