The authors describe a case of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) with recurrent neurological complications. A 24-year-old man experienced subacute bilateral visual loss. Based on the characteristic findings in funduscopy and fluorescin angiography and after the exclusion of the differential diagnoses, APMPPE was diagnosed. During the course of the disease recurrent episodes of meningitis and encephalitis occurred when tapering of prednisone was attempted. Secondary to encephalitic lesions, the patient developed partial epileptic seizures, which made an anticonvulsive medication necessary. The authors considered a chronic sinusitis to be an aetiological factor of the underlying autoimmune process. Due to the complicated course of APMPPE, they decided to start long-term immunosuppressive therapy with azathioprine under which the patient remained stable and prednisone could be tapered successfully. Neurological complications of APMPPE are rare. Nevertheless, this case demonstrates that long-term immunosuppressive treatment might be necessary to prevent recurrent neurological complications in some cases.