Hemolytic uremic syndrome is a multisystem disease that can affect central nervous system in up to 50% of cases. Central nervous system involvement can be clinically severe and its pathogenesis is not yet fully understood. Various magnetic resonance imaging findings, on conventional sequences, documenting the involvement of deep gray-matter structures, have been described. Diffusion-weighted imaging features of brain lesions have been reported only in 2 cases, but the potential role of this technique has not been considered yet. We describe a 19-month-old child affected by hemolytic uremic syndrome with basal ganglia lesions documented by diffusion-weighted imaging, with a 42-day neuroradiological follow-up and a 6-month clinical follow-up. In our case, diffusion-weighted imaging was more sensible in detecting the affected brain areas compared to T1, suggesting that reduced apparent diffusion coefficient values in the acute phase could reliably identify irreversible brain lesions in hemolytic uremic syndrome patients.