Background: Castelman’s disease (CD), also known as angiofollicular lymph node hyperplasia, describes a rare group of diseases manifesting with enlarged lymph nodes and various inflammatory symptoms. The association between Castelman’s disease, paraneoplastic pemphigus and bronchiolitis obliterans has been described in literature and is depicted thoroughly in this case. Case Presentation: We present a case of severe bronchiolitis obliterans developing in a 17-year old female with paraneoplastic pemphigus and unicentric Castelman’s disease. Conclusion: Surgical resection of unicentric Castelman’s disease remains the treatment of choice due to its efficacy in preventing recurrence of associated morbidity caused by bronchiolitis obliterans and paraneoplastic pemphigus.