Moyamoya disease is an idiopathic disease characterized by the progressive stenosis and collateral development of the distal internal carotid arteries. In this disease, several collateral vascular structures develop following stenosis and occlusion. The ivy sign is a characteristic Magnetic rezonance imaging (MRI) finding frequently encountered in patients with moyamoya. It can be observed both in post contrast T1-weighted images and Fluid attenuated inversion recovery (FLAIR) images. While this sign manifests in the form of contrasting on the cortical surfaces due to the formation of leptomeningeal collateral development and increased numbers of pial vascular webs on post contrast images, in FLAIR images it originates from the slow arterial flow in the leptomeningeal collateral vascular structures. In this case, we presented the Digital subtraction angiography (DSA) signs of moyamoya disease and "ivy sign" in MRI and its development mechanism in a 16 years old female patient.