AbstractThe co‐existence of idiopathic hemosiderosis and celiac disease is Lane‐Hamilton Syndrome. This is a rare condition with only a few dozen cases reported to date. Its clinical presentation typically involves hemoptysis that can be life‐threatening in the acute phase. We report the uncommon case of the development of idiopathic pulmonary hemosiderosis almost a decade after the diagnosis of celiac disease. Delayed diagnosis led to recurrent episodes of large volume hemoptysis despite immunosuppressive therapy due to ongoing ingestion of gluten. High doses of glucocorticoids accompanied by a cell cycle inhibitor mycophenolate mofetil were required for treatment. A strict gluten free diet is vital to control the disease. We highlight the importance of identifying this syndrome and definitive treatment, including avoidance of dietary triggers in addition to conventional immunosuppressive therapy.