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Hypocomplementemic urticarial vasculitis syndrome presenting with bilateral scleritis. Hypocomplementemic urticarial vasculitis syndrome (HUVS) is a rare autoimmune disorder characterised by recurrent urticarial lesions and acquired hypocomplementemia with systemic manifestations. The authors present the case of a 70-year-old man who presented to the ophthalmology clinic with bilateral scleritis and ocular hypertension. He was diagnosed with HUVS after a 6-month period of bilateral scleritis, vestibulitis, significant weight loss, mononeuritis multiplex and recurrent urticarial vasculitis with pronounced persistent hypocomplementemia and the presence of anti-C1q antibodies. Disease control was eventually obtained with mycophenolate and prednisolone.