Wegener's granulomatosis is a life-threatening condition characterized by the triad of necrotizing granulomatous inflammation of the respiratory tract, vasculitis, and glomerulonephritis. During early stages of the disease, some patients may present with only one or two components of the triad. Other patients may be affected by the limited form of Wegener's which lacks the renal involvement. We report an 18-year-old female who initially presented with bilateral dacryoadenitis. A lacrimal gland biopsy was consistent with Wegener's granulomatosis. A few months later she developed glomerulonephritis and responded well to cyclophosphamide treatment. Wegener's granulomatosis remains a diagnostic challenge and is frequently underdiagnosed. Early diagnosis is crucial in saving many lives because the disease is potentially curable with cytotoxic therapy. Dacryoadenitis may be one of the initial presentations of Wegener's granulomatosis.