Abstract: Neurofibroma has been one of the most common clinical manifestations in neurofibromatosis type 1, among which about 8%-13% has risk of malignant transformation. Previous researches suggest that angiogenesis plays an important role in the progression and malignancy of neurofibroma, while the anti-angiogenesis drugs have also demonstrated their potential clinical value in neurofibroma. In this article, the blood supply characteristics of neurofibroma, the mechanism of angiogenesis, and the research progress of anti-angiogenesis targeted therapy in neurofibroma are reviewed.