A 40-year-old woman presented to the eye clinic with painless swelling of her right lower eyelid of 2 years' duration. Examination revealed severe loss of vision in the right eye with hypoglobus and exotropia. CT showed a diffuse right orbital infiltrative lesion with extension to the brain parenchyma. Histopathology confirmed the diagnosis of orbital marginal zone B-cell lymphoma, which is an indolent type of lymphoma that can be associated with systemic lymphoma, but the intracranial association has not been previously described.