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OBJECTIVE: We report a patient with prolonged neurological complications in a patient with Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE). BACKGROUND: APMPPE is a rare, presumably autoimmune ophthalmologic disorder of the group of the white dot syndromes. Due to retinal vasculitis, fundoscopy reveals whitish coloured lesions, which become clinically overt in terms of reduced vision. Neurological complications and a malignant course of APMPPE are rare. DESIGN/METHODS: A 24-year old man with acute visual loss was diagnosed with APMPPE. Medical history was unremarkable apart from chronic sinusitis maxillaris. Ten days later, he presented with severe headache and meningism. Initial lumbar puncture revealed a lymphocytic inflammation with an elevated white cell count of 29/µl. RESULTS: The patient was diagnosed of having APMPPE associated meningitis. During the course of the disease, he presented with recurrent partial epileptic seizures including speech arrest and transient right-sided paraesthesia under prednisone tapering. Cerebral MRIs revealed several encephalitic lesions. Anticonvulsive therapy with carbamazepine was commenced. No distinct recommendations concerning long-term immunosuppressive therapy of APMPPE exist due to lack of case presentations in the literature so far. Steroids are treatment of first choice resulting in considerably good outcome in most cases. In view of recurrent neurological complications under prednisone tapering we initiated an immunosuppressive therapy with azathioprine under which the patient remained stable up to now. A chronic sinusitis maxillaris, which we considered an underlying cause or trigger of the autoimmune process, was subject to surgical intervention. CONCLUSIONS: Recurrent meningoencephalitis is a rare and severe complication of Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE). Even though long-term outcome is favourable in the majority of cases, severe and even fatal courses have been described and might require aggressive immunosuppressive treatment.