We describe the anesthesia management of a 12-year-old girl, diagnosed with fibrodysplasia ossificans progressiva (FOP), who presented with a submandibular abscess. FOP is a rare, inherited disorder with heterotopic bone formation and progressive musculoskeletal disability. This disability ultimately confines patients to a wheelchair. Minor trauma following dental treatment may lead to ankylosis of the jaw. Subsequent to this disability, which resulted in poor dental hygiene, our patient developed a dental abscess. This spread along the mandibular margin and under the tongue. She presented with an impending airway compromise in an already difficult situation. The options for airway management in a child with limited mouth opening are discussed.