Leprosy is a chronic infectious disease caused by Mycobacterium leprae. Erythema nodosum leprosum (ENL) is an immune complex-mediated type 2 leprosy reaction, and characterized by tender erythematous nodules involving the skin and peripheral nerves. In our case, 78-years-old man had weakness of extremities along with fever, arthralgia, numbness, and malaises from one month before visiting our hospital. He has several ulcerative plaques on the trunk, painful tender subcutaneous nodules on post-auricular area, and facial palsy. Eyebrow and eyelash alopecia were seen. Glove and stocking anesthesia was also evident. Both greater auricular nerves were thickened without tenderness. In histopathologic examination, neutrophilic infiltration diffusely scattered around blood vessels. The acid fast bacillus test showed bacillus and M. leprae PCR was positive. PGL-1 antibody level was 80. At presentation, he did not recognize that he had been diagnosed with leprosy in 1989. He arbitrarily discontinued the multiple drug therapy (MDT) of rifampin, dapsone and clofazimine a few months ago. The patient’s condition had improved with methylprednisolone administration. MDT had been maintained for about 12 months. With this case, we highlight the unusual presentation of lepromatous leprosy in erythema nodosum leprosum reaction and the need to exclude other infectious diseases after arbitrarily discontinued MDT.