The diagnosis and treatment of patients with tracheal tumors is challenging due to the low incidence of these tumors. A precise diagnostic work-up and experience is necessary to distinguish between patients amenable to surgery and nonsurgical patients. An individualized treatment concept should be established for each patient in a multidisciplinary team and ranges from interventional removal of benign lesions to multimodal strategies in patients with extended malignant tumors. Early diagnosis despite unspecific symptoms and complete resection are crucially important to achieve long-term survival.