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Bilateral disk edema in a case of retinitis pigmentosa can cause diagnostic confusion and delay in treatment. Existing literature shows few such cases where inflammatory pathology is postulated. A battery of tests may be required to reach a definitive diagnosis. Here, we will report a case of retinitis pigmentosa with bilateral disk edema due to malignant hypertension. Even in the presence of high systemic blood pressure, diagnostic dilemma can occur in such cases due to overlapping clinical features like arterial attenuation, decreased vision due to optic atrophy, and macular edema. Hence, other differential diagnoses like inflammatory causes and intracranial pathology should be carefully ruled out.

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