Neuroretinitis is a form of optic neuropathy typically characterised by acute unilateral visual loss with optic disc oedema and macular exudates arranged in a star figure around the fovea (complete or partial star). The purpose of this study was to determine the final diagnosis of patients referred with a diagnosis of neuroretinitis. A retrospective study of 40 patients found only 65% with a final diagnosis of neuroretinitis. This diagnosis was based on a comprehensive ocular and systemic history and review of medical systems. The remaining 35% (14 patients) had optic disc oedema with macular star (ODOMS) that had been mistaken for neuroretinitis. Of these patients 42.8% were found to have a previously unknown malignant systemic hypertension in association with bilateral ODOMS. In the cases with true neuroretinitis, 96.1% had unilateral involvement. This study highlights the importance of differential diagnosis for suspected neuroretinitis, especially when the signs are in both eyes, to avoid inappropriate work up and management and ensure early appropriate treatment. Clinicians need to be aware that ODOMS associated with unknown malignant hypertension can masquerade as neuroretinitis.