Background: Endogenous Candida endophthalmitis (ECE) is a rare but sight-threatening disease. Patients with ECE present with various clinical signs and symptoms, which can complicate the diagnosis. The aim of this report was to describe the characteristic features of ECE on optical co-herence tomography (OCT) and OCT angiography (OCTA). Another objective was to demon-strate the outcomes of treatment and to diagnose macular complications caused by intraocular inflammation. Case presentation: A 41-year-old woman with a history of acute intermittent por-phyria presented with a progressive vision loss in her left eye. Left-eye OCT revealed findings consistent with a fungal etiology, which was confirmed by the culture of swabs collected from a central vein catheter. The outcomes of intravenous fluconazole treatment were not satisfactory, and the patient developed recurrent attacks of porphyria, suggesting a porphyrogenic effect of systemic antifungal therapy. Repeated intravitreal injections with amphotericin B led to a gradu-al regression of inflammatory lesions. However, follow-up examinations revealed active macular neovascularization on both OCT and OCTA. The patient was administered intravitreal bevaci-zumab. Conclusions: This case highlights the importance of OCT and OCTA as valuable noninva-sive imaging techniques for the identification of ECE, the monitoring of its clinical course, and the diagnosis of macular complications.