We report a rare case of peripheral ulcerative keratitis (PUK) associated with Behçet's disease. A 34-year-old female presented with right ocular pain persisting for 8 months. The patient had been treated for previously diagnosed Behçet's disease. The corrected visual acuity was 20/32 in the right eye. Ocular examination revealed mild conjunctival hyperemia and a deep ulcerative lesion with perilesional haziness in the peripheral cornea. Autoimmune screening and corneal culture were negative. One month following treatment with topical and systemic immunosuppressants, the symptom had resolved. Slit-lamp examination showed a healed corneal lesion, and visual acuity improved to 20/20. Despite its rare occurrence, PUK can develop in patients with Behçet's disease. Therefore, patients presenting PUK require examination for Behçet's disease using a systemic evaluation.