Retinal angioma associated with von Hippel-Lindau disease

Von Hippel-Lindau disease is a multi-system disorder that can produce hamartomas (benign tumour-like nodules) of the eyes, skin and nervous system. Retinal capillary angioma is a common ocular association of this congenital phakomatosis that may result in blinding sequelae, if not managed appropriately. We present a case of retinal angioma associated with von Hippel-Lindau disease and discuss the ocular and systemic signs, diagnosis and management. The optometrist is of particular importance in screening for this disorder, as it is often first detected in a routine eye examination.