PURPOSE OF REVIEW: To discuss peripheral ulcerative keratitis, with a focus on the evaluation and management of associated systemic diseases. RECENT FINDINGS: Peripheral ulcerative keratitis (PUK) is a sight-threatening condition that is often defined by the presence of a crescent-shaped area of peripheral corneal thinning, an epithelial defect, and an inflammatory corneal infiltrate. It is highly associated with rheumatoid arthritis, systemic necrotizing vasculitides like granulomatosis with polyangiitis, and collagen vascular diseases like systemic lupus erythematosus. Undertreated PUK carries a risk of vision loss and premature death. SUMMARY: Multidisciplinary collaboration between the ophthalmologist, rheumatologist, and other consultants is required. Early and aggressive steroid-sparing therapy should be considered in cases due to noninfectious systemic disease.