Hydrancephaly is a rare condition where the brain's cerebral hemispheres are absent and the cranial vault is filled with fluid. It results from occlusion of the bilateral internal carotid or middle cerebral arteries, leading to liquefaction of brain tissue in the affected arterial territories while sparing structures supplied by the posterior circulation. Diagnostically, hydrancephaly is characterized by the absence of the cortical mantle with normal brain parenchyma replaced by liquefied tissue lined by leptomeninges, while the cerebellum and brain stem are usually intact. The prognosis is poor, with most infants dying within the first year of life.