Cyclophosphamide (CYC) is a classical drug for the treatment of severe granulomatosis with polyangiitis (GPA). However, a considerable number of patients are resistant to standard treatment and it can show substantial toxicity. Therefore, alternative agents should be considered in refractory cases. We report the successful use of rituximab (RTX) in a 70-year-old patient diagnosed of GPA who developed ophthalmoplegia, an uncommon complication of GPA. The patient also had upper and lower respiratory tract involvement and kidney manifestations with good prognosis. We review the causes of ophtalmoparesis and the treatment with RTX in GPA.