Paraneoplastic pemphigus(PNP) is a rare, life-threatening complication of neoplasm which presents with painful stomatitis and polymorphous skin lesions. Successful diagnosis of PNP can lead to the diagnosis of underlying malignancy and the proper treatment of it. Herein, we report a case of PNP associated with follicular lymphoma treated with chemotherapy including rituximab, with complication of bronchiolitis obliterans as an educational case. 44-year old woman presented with severe erosive stomatitis and maculopapular rash on the whole body. After a presumptive diagnosis of Stevens-Johnson syndrome, 1.5mg/kg of methylprednisolone was administered but stomatitis did not respond. Skin biopsy showed interface dermatitis with subepidermal blister with linear C3 deposition on dermoepidermal junction. On indirect IF, IgG deposition on the mouse bladder and lung epithelium were detected. The diagnosis of PNP was made. On subsequent work up, multiple enlargements of lymph nodes were found and follicular lymphoma was diagnosed. After R-CHOP(rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) chemotherapy, complete remission of lymphoma was achieved. Skin lesions and stomatitis were remarkably improved and maintained without recurrence for 6 months. However her respiratory function is deteriorating due to bronchiolitis obliterans, which is a well-known complication of PNP.