The 10 year old female patient presented with sustained hypertension, headaches, and vomiting for several weeks. She had no signs of hypertensive retinopathy or papilledema. However, in the right eye, a small, white-yellowish nodular lesion in the retinal periphery was found. Luoresceinangiography showed early and late leakage and marked hyperfluorescence compatible with retinal hemangioblastoma. A heterozygous mutation 488T>A (Leu163His) in exon 3 of the VHL gene was identified (methods unspecified). Subsequently, bilateral pheochromocytomas were found. After surgical resection of the pheochromocytomas the patient was asymptomatic. No family history was mentioned.