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Delayed diagnosis and a need for multiple biopsies were reported in numerous cases. Two cases of skull base IgG4-RD from the authors' institution show the variable presentations of this disease. More invasive surgical biopsies were required in both cases, and corticosteroid treatment led to significant clinical improvement. IgG4-RD is an uncommon condition with an increasing body of reported cases that can affect the skull base and calvarium and should be in the differential diagnosis, because delay in diagnosis and treatment may be common. IgG4-RD