This case report aims to highlight the complexity of diagnosing and treating pediatric anti-NMDA receptor encephalitis, underscore the importance of early intervention with multimodal therapy, and discuss the unique aspects of managing severe and novel presentations of this condition in a very young patient.

Anti-NMDA receptor encephalitis presents a formidable challenge in pediatric neurology, characterized by diverse neuropsychiatric manifestations and a critical need for prompt, aggressive treatment. This case report is distinguished by its focus on a uniquely very young patient with severe disease presentation, detailing an innovative treatment regimen that underscores the necessity of early and aggressive intervention. It adds significantly to the limited pediatric literature, showcasing a successful outcome despite formidable odds.

A 2-year-old African American female with a history of seizures since July 19, 2021, presented with generalized tonic-clonic seizures, altered mental status, choreoathetoid movements, tachycardia, hyperthermia, and leukocytosis. The diagnostic process revealed high titers of NMDA receptor antibodies in the cerebrospinal fluid, leading to a diagnosis of anti-NMDA receptor encephalitis.