We describe a 20-year-old Japanese woman with anaplastic large cell lymphoma in whom primary tracheal involvement was associated with life-threatening airway obstruction. After debulking surgery, the patient was successfully treated with chemotherapy and radiotherapy. A total of 28 cases of primary tracheal lymphoma were reviewed. Common clinical features included predominance of localized disease, respiratory symptoms resembling acute asthma, absence of hemoptysis, and rapid progression of tracheal stenosis. Various histological subtypes of primary tracheal lymphoma exist and are likely to determine prognosis.