Background: Reported cases of knife injuries to the head in children are rare.Here we describe a case of pediatric penetrating brain injury by knife (PPBIK) and review literature. The patient's parents were informed that non-identifying information from the case would be submitted for publication, and they provided consent. Case Presentation: A 3-year-old boy presented to our emergency department with a knife penetrating the front of his head during play. Neurologic examination was normal. Radiograph in lateral view demonstrated a metallic knife entering frontal bone. Computed tomography (CT) scan showed small hemorrhage around the foreign body recognized, accompanied by small pneumocephalus. The knife was surgically removed and the dura was closed. He was discharged home after the surgery with normal neurological status. Conclusion: Only four cases of PPBIK have been reported in the literature. The adequate management of these types of injuries requires a correct neuroradiological evaluation.