Purpose The aim of this study was to report the presence of focal limbal stem cell deficiency (LSCD) in 2 cases of Turner syndrome. Methods This is a case review. Results A 25-year-old woman with a history of Turner syndrome diagnosed at 15 years of age was referred for "advanced chronic bilateral phlyctenular disease." The patient's visual acuity at presentation was 20/25-2 in the right eye and 20/30-2 in the left eye. Slit-lamp examination of both eyes showed inferior conjunctivalization of the corneal epithelium consistent with LSCD. The patient was started on topical cyclosporine 0.05%. A 23-year-old woman with the Turner syndrome was referred for rosacea keratitis. An ocular assessment revealed the visual acuity at presentation to be 20/25 in the right eye and 20/30 in the left eye. Slit-lamp examination of both eyes showed significant conjunctivalization with epithelial scarring and opacity. The patient was started on erythromycin ointment before bedtime, artificial tears, and warm compresses. Conclusions This study reports the first cases in the literature describing the coexistence of LSCD and Turner syndrome. Management of any ocular surface inflammation is important to prevent the progression of LSCD.