Angiomatosis retinae is a rare disease chara3terized by the immense dilatation and tortuosity of some retinal vessels and the development of angiomas. Von Hippel described firstly this ocular disease in 1895. It was also von Hippel who established this ocular change as a clinical entity in 1904. This disease was then associated with the name of von Hippel. The complete concept of angiomatosis of the retina as part of the general systemic vascular disease was established firstly by the classical research of Lindau in 1926-1927. The case reported here was a 38 years old Taiwanese housewife who suffered from progressive loss of left eye vision one year ago before her admission. Total loss of left eye vision occured two months prior to her admission. She was admitted for detail examination. Immense dilatation of veins associated with several large neurysms were discovered in her right fundus. Whitish exudation was also noted. Systemic examination including neurological, radiological and cerebroangiography revealed no intracranial implications. Patient discharged voluntarily before any management was given. She died four days later after the discharge. The cause of death was not clarified.