The recognition of pulmonary manifestations of inflammatory bowel disease (IBD) is important as these diseases can be confused with infectious etiologies (e.g., tuberculosis or fungal infection) and, as a result, may unnecessarily delay institution of appropriate therapy (e.g., infliximab). Furthermore, they are a source of morbidity that may be overlooked and, like other IBD-related pathologies, are often responsive to treatment with corticosteroids, immunomodulators, or biologic therapies. The purpose of this paper is to describe the cases of six children at a single institution with differing presentations, treatments, and responses to treatment of their IBD-related lung disease to improve recognition of this uncommon process.