We examined the fiber organization of the brain in three patients with unilateral polymicrogyria (PMG) using diffusion tensor imaging (DTI) in combination with functional magnetic resonance imaging (fMRI). DTI revealed altered fiber tract architecture in patients with PMG. Long projection fibers, such as the corticospinal tract, were reduced the most, whereas long association fibers were less affected. The diminution of the fiber tracts was relevant to the loss of functionality of the PMG-affected cortex. Our preliminary study suggests that the combination of DTI and fMRI reinforces the clinical assessment of functionality in PMG.