Methemoglobinemia can be caused by dapsone toxicity. We report a case dapsone induced methemoglobinemia unresponsive to methylene blue successfully treated by exchange transfusion. A 52-year-old male ingested a handful of dapsone. He presented with severe peripheral cyanosis in lips and fingertips and his methemoglobin level was found to be 21.9%. After admission, methylene blue (1%) at 1 mg/kg was injected each time peripheral cyanosis and rising serum methemoglobin occurred. Despite methylene blue therapy, the patient‘s methemoglobin level continued to fluctuate. Five days after the injections of methylene blue, many Heinz bodies were visualized in the peripheral blood, suggestive of hemolytic anemia occurrence. By hospital day 6, serum methemoglobine levels were elevated and not measurable (> 50%) and the patient was constantly in a semi-comatose mental state. An exchange transfusion carried out by utilizing 6 units of packed red blood cells and 4 units of fresh frozen plasma was performed. The patient's methemoglobin levels were subsequently kept up below 20% and his peripheral cyanosis receded. Physicians should recognize the important role of exchange transfusion in refractory dapsoneinduced methemoglobinemia.