Falcine meningioma in Von Hippel–Lindau disease: An unusual association

BACKGROUND: Von Hippel–Lindau (VHL) disease is an autosomal dominant condition characterized by formation of multiple benign and malignant tumors. In this disease supratentorial lesions are rare and no falcine meningioma has been previously reported. Differential diagnosis is very difficult and the histopathological examination is the definitive method for diagnosis. CASE DESCRIPTION: A patient with VHL underwent a suboccipital craniotomy for removal of cerebellar hemangioblastoma and after 2 years magnetic resonance imaging (MRI) showed an iperintense solid mass located at posterior part of the falx. Histological diagnosis revealed meningioma. CONCLUSION: The only case in the literature of falcine meningioma in a patient with Von Hippel–Lindau disease, discovered during radiological follow-up, is described and a surgical management is proposed.