Holoprosencephaly is the most common developmental disorder of the prosencephalon. We present a case of alobar holoprosencephaly with cortical migration anomalies, a large midline mass, a monooptic nerve, and no dorsal cyst. Diffusion tensor tractography showed markedly abnormal supratentorial and posterior fossa white matter fibers, along with an enlarged hippocampal commissure across the dorsal midline cerebrum and a monooptic nerve projecting toward the cerebellum. Fiber tracking revealed information about white matter composition and organization not evident with conventional MR imaging.