Advanced VHL eye disease correlates with occurrence of central nervous system and systemic lesions. Spectrum of vhl gene mutation in the patients corresponds to that of the general VHL population. Pars plana vitrectomy in advanced VHL eye disease can improve or preserve visual function, but postoperative progression of ocular VHL disease can be accelerated in cases where retinotomy is performed.

A new case with OMD is added to preceding reports. The mfERG and OCT tests are important in detection of OMD patients. It can help in differential diagnosis of amblyopia, optic nerve diseases and non-organic visual disorders.