This study assessed diffusion abnormalities of the optic nerve (ON) in giant cell arteritis (GCA) patients with acute onset of visual impairment (VI) using diffusion-weighted magnetic resonance imaging (DWI). DWI scans of GCA patients with acute VI were evaluated in a case-control study. Two blinded neuroradiologists assessed randomized DWI scans of GCA and controls for ON restricted diffusion. Statistical quality criteria and inter-rater reliability (IRR) were calculated. DWI findings were compared to ophthalmological assessments. 35 GCA patients (76.2 ± 6.4 years; 37 scans) and 35 controls (75.7 ± 7.6 years; 38 scans) were included. ON restricted diffusion was detected in 81.1% (Reader 1) of GCA scans. Localization of ON restricted diffusion was at the optic nerve head in 80.6%, intraorbital in 11.1% and affecting both segments in 8.3%. DWI discerned affected from unaffected ON with a sensitivity, specificity, positive and negative predictive value of 87%/99%/96%/96%. IRR for ON restricted diffusion was κinter = 0.72 (95% CI 0.59–0.86). DWI findings challenged ophthalmologic diagnoses in 4 cases (11.4%). DWI visualizes anterior and posterior ON ischemia in GCA patients with high sensitivity and specificity, as well as substantial IRR. DWI may complement the ophthalmological assessment in patients with acute VI.