The aim of this study was to evaluate a patient with von Hippel-Lindau (VHL) syndrome and to discuss the clinical manifestations. A 25-year-old woman presented to our clinic with blurred vision for 2 months. Best corrected visual acuity in right eye was 20/50, and fundoscopic examination revealed juxtapapillary endophytic retinal capillary hemangioma. Examination findings on left eye were normal. On optical coherence tomography, exudation was prominent on macula. History revealed cerebellar operation because of cerebellar hemangioblastoma. On abdominal ultrasonography, liver hemangiomas and pancreatic cysts were seen, confirmed by abdominal computerized tomography (CT). Abdominal CT imaging also revealed nutcracker phenomenon. Transthoracic echocardiography showed atrial septal aneurysm without shunt. The patient refused ocular treatment. At 3-month control, ophthalmic findings were the same. To our knowledge, this is the first case of VHL disease reported to be associated with nutcracker phenomenon and atrial septal aneurysm. Systemic evaluation and regular follow-up should be recommended to subjects with VHL disease.