Purpose <p>To measure the retinal vessel oxygen saturation alteration with retinal oximetry (RO) in patients with acute zonular occult outer retinopathy (AZOOR) and compare these findings with retinitis pigmentosa (RP) and controls.</p> Methods <p>Prospective observational study examining 189 eyes of 98 patients: 12 eyes of 6 AZOOR patients (1♂5♀; 48.5 ± 19.2 years (mean ± SD) were compared to 77 eyes from 39 patients with RP (15♀ 24♂; 45.6 ± 14.7 years) and 100 eyes from 53 controls (31♀ 22♂; 40.2 ± 13.4 years). Main outcome measures were the mean arterial (A-SO₂; %), venular (V-SO₂; %) oxygen saturation, as well as their difference (A-V SO₂; %) measured with the oxygen saturation tool of the Retinal Vessel Analyzer (IMEDOS Systems UG, Germany).</p> Results <p>Eyes affected by AZOOR differed significantly from both RP and control eyes in terms of retinal oxygen metabolic parameters. Compared to controls, AZOOR eyes showed significantly higher A-SO₂ and V-SO₂ values (<i>p</i> = 0.031 and <i>p</i> = 0.044, respectively), while V-SO₂ was significantly lower in AZOOR compared to RP eyes (<i>p</i> = 0.035). A–V SO₂, which reflects retinal oxygen consumption, was significantly reduced only in RP eyes, but not in AZOOR eyes, when compared to controls.</p> Conclusion <p>Assessments of retinal oxygen metabolism might contribute to a correct diagnosis and a better understanding of the pathogenesis of AZOOR and RP.</p>