Angiocentric T-cell lymphoma (lymphomatoid granulomatosis) may present with prominent central nervous system (CNS) findings with variable radiographic features. We describe a patient who presented with multiple cranial nerve palsies involving the left optic nerve, left facial nerve, left ocular motor nerves, and bilateral acoustic nerves. Enhancement of the right temporal meninges and a cavernous sinus mass were noted on magnetic resonance (MR) scan. A right temporal craniectomy and meningeal biopsy were performed. Meningeal biopsy revealed atypical angiocentric granulomatous lymphoid infiltrates without associated necrosis, giant cells, or granuloma formation. Morphologic and T-cell, receptor gene rearrangement findings were diagnostic of an angiocentric T-cell lymphoma. Retrobulbar optic neuropathy and multiple cranial nerve palsies may be the presenting features of angiocentric T-cell lymphoma. The neurologic and unique radiographic changes in our case expand the previously reported findings in CNS angiocentric T-cell lymphoma.