A 34-day-old male presented with a rare neonatal ruptured aneurysm of the distal posterior inferior cerebellar artery manifesting as a 10-day history of enlargement of head circumference. Magnetic resonance imaging revealed hydrocephalus and a round infratentorial enhanced lesion which compressed the medulla. Left vertebral angiography demonstrated an aneurysm on the telovelotonsillar segment of the left posterior inferior cerebellar artery. Ventriculoperitoneal shunt emplacement and proximal artery clipping were performed. The cerebrospinal fluid was bloody, suggesting aneurysm rupture had caused hydrocephalus. His postoperative course was uneventful, and neurological and developmental findings were normal 7 months later. Present neuroimaging, surgical, and neuroanesthesiology techniques allow successful surgical intervention in cases of neonatal ruptured aneurysm.