Background. Foster Kennedy syndrome (FKS) is described as ipsilateral optic atrophy and contralateral papilledema from an intracranial mass. FKS is uncommon manifestation of Neurofibromatosis type 2 (NF2), which is gen erally presented with hearing loss and tinnitus. Case report. In this report we present a 26 year old female with the atypical presentation of NF2. First symptoms were progressive vision loss and cognitive dysfunction. Ophthalmological examination reveale d right sided papilledema and left sided optic atrophy. Magnetic resonance imaging (MRI) of the brain revealed bilateral vestibulocochlear schwannoma and three intracranial meningiomas, involving the parafalcine region and the olfactory groove. Whole spine MRI showed one intramedullary tumor at C1 C2 level, multiple spinal canal nodules in cervico dorsal regions and one Th12 L2 extramedullary tumor. Based on clinical and imaging findings the diagnosis of neurofibromatosis type 2 was established. The patient underwent surgical resection of giant parasagittal meningioma, subtotal resection of the olfactory groove meningioma and total resection of Th12 L2 meningioma. Six months after brain surgery, she underwent Gamma knife radiosurgery for remnant frontobasal meningioma and for both vestibulocochlear schwannomas. Despite the combined treatment of intracranial lesions, only an insignificant vision improvement was achieved. Conclusions. FKS can be the presenting symptom of NF2. Early detection and treatment of o phthalmologic manifestations of NF2 may prevent amblyopia development.