We report a neonate with brainstem disconnection. Only five similar cases have previously been described, the longest survival was 7 weeks. The newborn exhibited muscular hypertonia, absent suction, insufficient breathing, and seizures. Magnetic resonance imaging showed a disconnection between the upper pons and the medulla oblongata and cerebellar hypoplasia. The basilar artery was not visible. These neuroimaging findings are clearly different from other midbrain or hindbrain malformations with cerebellar hypoplasia. This pattern and a previously reported autopsy point to a malformation, not a disruption.