Introduction:Small artery disease caused by neutrophils and immune-mediated is known as leucocytoclastic vasculitis (LCV). Clinically, it manifests as palpable, asymptomatic purpuric papules on the limbs. Ocular manifestation is rare. Here, we describe a case of peripheral ulcerative keratitis (PUK) associated with LCV.Case presentation:A 59-year-old man was referred to the hospital with blurred vision due to corneal perforation in his left eye. He complained of itchy nodules on his hands and lower legs for 15 years and the skin biopsy of the back of his hand revealed LCV 6 years ago, which suggested erythema elevatum diutinum. The patient was under treatment with anti-inflammatory and immunosuppressive drugs and physical features of LCV seen in him included erythema on his hands and legs. After receiving conjunctival flap covering surgery, the corneal perforation was resolved. Conjunctival flaps covered cornea that limited his vision to hand motion. Six months later, he was referred to our clinic again because of pain, redness, photophobia, and tearing in the right eye, presenting with PUK. Necrotic tissue was removed during surgery, which also included a conjunctival flap covering procedure. Following surgery, the symptoms were reduced, and the postoperative eye condition remained stable.Conclusion:To our knowledge, it is the first case of PUK secondary to LCV which was diagnosed 6 years ago. This case demonstrates that PUK associated with LCV can be successfully treated by surgical interventions.