Abstract
BACKGROUND: Stroke-like migraine attack after radiation therapy (SMART) syndrome is a rare, episodic neurological condition characterized by headache and unilateral cortical deficits following remote cranial radiation therapy. It is more frequently reported in adults, while pediatric cases remain exceedingly rare due to the latency period between radiation therapy and onset, which can span years to decades. Diagnosis is challenging due to overlapping symptoms with conditions like ventriculoperitoneal shunt (VPS) malfunction. OBSERVATIONS: The authors report the case of an 11-year-old boy with SMART syndrome following radiation treatment for a resected pineal region nongerminomatous germ cell tumor and VPS placement. The patient presented with headache, vomiting, lethargy, and seizures, raising initial concern for shunt malfunction. MRI revealed left occipital and posterior temporal cortical FLAIR hyperintensity and abnormal leptomeningeal enhancement, consistent with SMART syndrome. Multidisciplinary evaluation ruled out shunt failure and tumor recurrence. A course of corticosteroids led to symptom resolution. A review of 15 pediatric cases highlights variability in latency, symptoms, and treatment, with most patients responding favorably. LESSONS: SMART syndrome, although rare, should be considered in pediatric patients with postradiation episodic neurological symptoms to avoid unnecessary interventions. Multidisciplinary collaboration is essential. Further studies are needed to establish standardized diagnostic and treatment protocols. https://thejns.org/doi/10.3171/CASE25189.