Abstract
Atypical teratoid/rhabdoid tumors (AT/RTs) of the central nervous system (CNS) are rare and aggressive, typically occurring in early childhood or infancy, with adult cases being extremely rare. These tumors are associated with the inactivation of the integrase interactor 1 (INI1) gene. The prognosis is poor, worsening significantly if metastasis is detected at diagnosis. While CNS tumors rarely metastasize to cervical lymph nodes, recent findings have shown that such dissemination is possible, challenging the previously held belief that CNS malignancies do not spread via the lymphatic system. Awareness of this potential pathway is crucial for early diagnosis and avoiding unnecessary treatments. We present a case of a young adult male patient with a primary CNS AT/RT who had presented with cervical lymph node metastasis.