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Hundreds of distinct epilepsy-causing genes have been identified.1 The vitamin B6-dependent epilepsies are a heterogeneous group genetic disorders due to incomplete formation, transport, or inactivation pyridoxal 5′-phosphate (PLP).2 ALDH7A1 , PNPO ALPL ALDH4A1 and more recently PLPBP implicated.3 Characteristic presentation includes neonatal onset encephalopathy seizures refractory first-line antiseizure medications (ASMs) followed by cessation dramatic improvement after administration pyridoxine PLP. Because initiation proper treatment is essential for seizure control, timely diagnosis critical optimizing clinical outcome. We describe the case 3-year-old boy who presented with progressively worsening starting at 14 months age was subsequently diagnosed phosphate homeostasis protein (PLPHP) deficiency via rapid genome sequencing during care hospitalization status epilepticus. He started on immediate activity has remained seizure-free. review diagnosis, management, outcome PLPHP deficiency, as well implications use testing in acute setting.