Acute necrotizing encephalopathy of childhood is a unique entity with bilateral gray and white matter involvement. The aim of this study is to explore whether the severity of findings on imaging scans is indicative of the prognosis and clinical outcomes for pediatric patients with acute necrotizing encephalopathy. A retrospective cross-sectional study was conducted on 42 patients diagnosed with acute necrotizing encephalopathy. A severity score based on MR imaging was computed for each patient, utilizing a point system determined by the existence of factors such as hemorrhage, cavitation, enhancement, diffusion restriction, and lesion location. The scoring was categorized into mild, moderate, and severe. Clinical outcomes were determined at the time of discharge and at follow-ups as mild disability, moderate disability, severe disability, and death according to the modified Rankin Scale. Associations were determined by Fisher's exact test, chi-square test, and one-way ANOVA. The study included 21 boys and 21 girls with a mean age of 71.5months. A statistically significant connection (P=0.027) was found between the severity score from MR imaging and the clinical outcome. A statistically significant relationship was also observed between diffusion restriction (P=0.008), cerebellar involvement (P=0.048), and an unfavorable clinical outcome. Additionally, individuals who experienced shock exhibited a correlation with adverse outcomes (P=0.01). In predicting the outcome of acute necrotizing encephalopathy, cerebellar involvement and presence of diffusion restriction were associated with worse clinical outcomes in our study. Developing a comprehensive MR-based severity score is crucial for improving diagnostic accuracy and patient outcomes. Our findings underscore the importance of including diffusion restriction and cerebellar involvement in the scoring system.