PURPOSE To describe a case of endogenously acquired Candida albicans chorioretinitis that was unresponsive to aggressive local and systemic therapy. METHOD Single retrospective case report and review of pertinent literature. A 42-year-old woman with metastatic colon cancer acquired endogenous C. albicans chorioretinitis secondary to total parenteral nutrition after a small-bowel resection. RESULTS A lesion involving the left fovea progressed despite systemic antifungal therapy with intravenous anidulafungin followed by oral fluconazole, intravenous Ambisome, and 11 total intravitreal injections of either Amphotericin B (5 mg each) or voriconazole (50-100 mg each). The patient underwent pars plana vitrectomy with subfoveal aspiration and removal of infectious material using submacular surgery, resulting in resolution of the infection. Postoperatively the patient's visual acuity was the same as before surgery (20/200 in the left eye). CONCLUSION When treating endogenous fungal chorioretinitis, attention should be paid to both systemic treatment that has good intraocular penetration and early local therapy with intravitreal antifungals for sight-threatening lesions. Surgical intervention may also be warranted.