Objective: To report a female patient with bilateral limbal stem cell deficiency (LSCD) due to primary adrenocortical insufficiency (PAI). Methods: Case report Results: A 40-year-old female patient had blurry vision, foreign body sensation, tearing, and photophobia for several years. On examination, corneal epithelial haze, surface irregularity, and superficial neovascularization were observed. There was a dull and irregular reflex from the conjunctivalized corneal surface. Medical history revealed that she had a diagnosis of PAI for 11 years and received hormone replacement (fludrocortisone acetate) therapy. With the clinical presentation and examination, the diagnosis was compatible with LSCD. Frequent ocular lubricant and topical steroid drops were initially started and topical cyclosporine treatment was planned for the long term. After 3 weeks, there was no corneal superficial neovascularization and epithelial haze, peripheral stromal haze was still observed. Conclusion: LSCD may rarely be associated with PAI. In patients with LSCD, systemic evaluation should be made to rule out PAI.