IgG4 related disease is an uncommon disease that presents with diverse clinical manifestations. Interestingly the disease is a great masquerade of many other autoimmune and malignant conditions. Rare occurrence and varied clinical phenotype makes this disease a challenging one for the clinicians. Here we present a case of IgG4 related disease in a 50 year old female, who initially presented with low grade fever, sicca symptoms, eyelid swelling with proptosis along with bilateral salivary gland enlargement. Final diagnosis was made from characteristic histopathological finding and markedly elevated serum IgG4 level.