International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties. Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. ABSTRACT Introduction:

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... Von Hippel-Lindau (VHL) disease is a complex and systemic entity that can be discovered by neurological complications. Its association with other serious malformative disease like nutcracker syndrome (NCS) was not described, and its complex presentation must not make some other serious problem easily missed or neglected. Case Report: A 27-year-old male patient with 3 intramedullary hemangioblastoma of six months of insidious evolution complicated by medullary compression. The lesions were completely removed with excellent neurological postoperatory outcome. Thinking of VHL disease, our investigations were expanded and revealed multiple vermian and cerebellar hemangioblastomas, renal masses, and multiple pancreatic cysts. In addition, an incidentally found of posterior NCS during the abdominal computed tomography (CT) done for VHL disease investigations. Our attitude to both neurosurgical and urological problems (the serious presentation of VHL disease with multiple locations of hemangioblastoma and right renal carcinomas) and for the missed symptomatic posterior nutcracker syndrome were discussed. Conclusion: The complex presentation of the VHL disease must not makesome other serious problems easily missed or neglected like renal carcinoma or nutcracker syndrome as seen in our case. AbstrAct Introduction: Von Hippel-Lindau (VHL) disease is a complex and systemic entity that can be discovered by neurological complications. Its association with other serious malformative disease like nutcracker syndrome (Ncs) was not described, and its complex presentation must not make some other serious problem easily missed or neglected. case report: A 27-year-old male patient with 3 intramedullary hemangioblastoma of six months of insidious evolution complicated by medullary compression. the lesions were completely removed with excellent neurological postoperatory outcome. thinking of VHL disease, our investigations were expanded and revealed multiple vermian and cerebellar hemangioblastomas, renal masses, and multiple pancreatic cysts. In addition, an incidentally found of posterior Ncs during the abdominal computed tomography (ct) done for VHL disease investigations. Our attitude to both neurosurgical and urological problems (the serious presentation of VHL disease with multiple locations of hemangioblastoma and right renal carcinomas) and for the missed symptomatic posterior nutcracker syndrome were discussed. conclusion: the complex presentation of the VHL disease must not makesome other serious problems easily missed or neglected like renal carcinoma or nutcracker syndrome as seen in our case.