Non-Hodgkin's lymphoma (NHL) is a rare tumor of the skull base. As the inci
dence of primary central nervous system (CNS) lymphoma has increased, atypi
cal presentations involving the skull or cranial base exclusively have been
reported. In immunocompetent patients with no previous history or predispo
sing factors, the diagnosis of primary NHL of the skull base may be delayed
. We present four cases of nasal and paranasal sinus NHL with both skull ba
se and intracranial involvement in immunocompetent patients. Clinicopatholo
gic correlation suggests that cranial base and intracranial involvement wit
h NHL represents advanced-stage primary sinonasal disease. Surgical biopsy
before definitive treatment is recommended. Radiation therapy provides loca
l control; adjuvant chemotherapy after primary radiation therapy may be req
uired for recurrent disease.