According to evidence in the literature occurrence of the Hippel Lindau disease changes from 1: 33000 - 1: 36000 (population frequency) to 1 in 39000 live births. Up to 20% of them have not family history. Hereditary predisposition of most patients with systemic angioreticulomatosis has patterns and attributes. Diversity of forms and infrequent cases of the Hippel Lindau disease complicate timely diagnosis. Untimely control of condition of patient with the Hippel Lindau disease increases the

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... bability of complications in surgical treatment. It can be the reason of early disability too. The presented clinical case reflects the need for a multidisciplinary approach to the management of patients with von Hippel-Lindau syndrome.