Background: Immunoglobulin G4 (IgG4) related disease is recently recognized autoimmune disease, characterized histologically by dense infiltration of numerous IgG4 positive plasma cells, storiform fibrosis. Isolated involvement of esophagus is very rare and only few cases of IgG4 related esophagitis and its radiological findings have been reported. Case report: 72–years–old male patient presented with history of painless and progressive difficulty in swallowing for 1 month. The pre–operative diagnostic assessment revealed a long segment tight fibrosed benign looking smooth stricture of distal esophagus with smooth nipple like projections and indentation over proximal end of stenotic segment. Conclusion: Benign looking radiological findings and high index of clinical suspicion for IgG4–related disease in cases of chronic worsening stricture and debilitating dysphagia may help in proper & early decision making.