A 73-year-old woman with a history of muscular weakness and dyspnoea of unknown etiology was referred to our Ophthalmology Department for dacryocystitis. Lacrimal sac biopsy revealed IgG4 plasma cell infiltration and systemic diagnosis was done based on this, allowing an appropriate treatment to be established. To our knowledge, this is the first reported case of IgG4-related dacryocystitis associated to aortitis.