The objective of the paper is to draw attention to an uncommon picture of Best s vitelliform dystrophy of the macula (BVD)complicated by a choroidal neovas- cular membrane (CNVM)and haemorrhage. In an 8-year-old boy where incidentally at the age of 5 years cystic changes in both maculas were found and a reduced central visual acuity (CVA)of the left eye,the CVA of the so far asymptomatic right eye suddenly deteriorated.In the right macula a vitelliform focus was present the lower half of which was formed by a vascularized scar with subretinal haemorrhage on the margin.In the left macula was a round chorioretinal scar with hyperplasia of the pigmented epithelium (RPE)and rem- nants of macular yellow.Fluoroangiographic examination (FAG)visualized classical CNVM,optical coherence tomography (OCT)ablation of the RPE.The electrooculo- gram (EOG)was bilaterally reduced,CVA of the right eye was 6/60,of the left eye 6/36.Examination of the mother revealed though the ophthalmological finding was normal,a significantly reduced EOG and in an older brother there was a small vitelliform focus in both maculae and reduced EOG with CVA of 6/6. Conclusion:CNVM with haemorrhage is a rare complication of BVD.This compli- cation markedly extends the polymorphism of findings,is a risk of the CVA and makes the diagnosis more difficult.EOG examination is decisive for the diagnosis of less common forms of BVD.

        Key words: Best ’s vitelliform dystrophy,choroidal neovascular membrane.