An 83 year old gentle man with history of recurrent urothelial cancer was treated with Nivolumab and NKTR every three weeks. After second cycle, he developed hoarseness, difficulty speaking, swallowing, generalized weakness, poor appetite. The patient was admitted to the hospital. Lab showed CK 4000 U/L, aldolase 55.6 U/L, acetylcholine receptor binding antibody 0.16 nmol/L, acetylcholine modulating antibody 45%, striate muscle Ab positive at 1:30720. EMG/NCV suggested generalized myopathy with irritable features suggestive of inflammatory/reactive process. Muscle biopsy showed skeletal muscle and fibroconnective tissue with focal perimysial chronic inflammatory cell infiltrate. Chest CT did not show any thymoma. Other autoimmune and paraneoplastic panel were unremarkable. Nivolumab related MG and myositis were diagnosed. Patient was started on methylprednisolone and plasmapheresis at the admission. However, he required intubation due to respiratory failure. He failed spontaneous breathing trials multiple times and required tracheostomy placement. Patient was also treated with pyridostigmine, cellcept, rituximab and tacolimus. He had received plasmapheresis and IVIG. Patient was finally discharged home hospice with tracheostomy.