Background <p>Baló’s concentric sclerosis (BCS) is a rare demyelinating disorder distinguished by its hallmark imaging feature of concentric layers of demyelination on magnetic resonance imaging (MRI). Despite its distinct radiological presentation, the pathogenesis and clinical significance of BCS remain poorly understood, making the reporting of such cases crucial for advancing knowledge about its diagnosis and management. Through this case report, we aimed to highlight the clinical and radiological features of BCS and emphasise the role of MRI in its early diagnosis and management.</p> Case presentation <p>We present a case of BCS characterised by a severe clinical presentation and a striking radiological profile. The patient exhibited profound neurological deficits, and a brain MRI was performed, demonstrating the hallmark concentric demyelination pattern. Diagnosis was based on clinical presentation and characteristic imaging findings. High-dose corticosteroid therapy led to significant clinical recovery and marked radiological improvement.</p> Conclusions <p>This case contributes to a deeper understanding of the pathophysiological processes behind BCS and underscores the importance of recognising its characteristic imaging features. Improved awareness may enhance diagnostic accuracy and influence therapeutic approaches for this rare condition.</p>