MRI in two children with moya-moya demonstrated low signal on T2-weighted images in the acute and subacute phases of ischaemia. Gradient-echo sequences, more sensitive to magnetic susceptibility, demonstrated these abnormalities better. Signal loss, due to temporary accumulation of iron, decreases progressively and disappears in the chronic stage of the disease. Diffusion-weighted MRI allows early detection of ischaemic lesions and can be used to monitor progressive spreading of the lesions. Magnetisation transfer maps provide sharp contrast, useful for demonstrating cortical atrophy.