The symptoms of carbon monoxide (CO) poisoning are nonspecific, ranging from dizziness and headache to unconsciousness and death. A German national guideline on the diagnosis and treatment of this condition is lacking at present.This review is based on a selective literature search in the PubMed and Cochrane databases, as well as on existing guidelines from abroad and expert recommendations on diagnosis and treatment.The initiation of 100% oxygen breathing as early as possible is the most important treatment for carbon monoxide poisoning. In case of CO poisoning, the reduced oxygen-carrying capacity of the blood, impairment of the cellular respiratory chain, and immune-modulating processes can lead to tissue injury in the myocardium and brain even after lowering of the carboxyhemoglobin (COHb) concentration. In patients with severe carbon monoxide poisoning, an ECG should be obtained and biomarkers for cardiac ischemia should be measured. Hyperbaric oxygen therapy (HBOT) should be critically considered and initiated within six hours in patients with neurologic deficits, unconsciousness, cardiac ischemia, pregnancy, and/or a very high COHb concentration. At present, there is no general recommendation for HBOT, in view of the heterogeneous state of the evidence from multiple trials. Therapeutic decision-making is directed toward the avoidance of sequelae such as cognitive dysfunction and cardiac complications, and the reduction of mortality. Smoke intoxication must be considered in the differential diagnosis. The state of the evidence on the diagnosis and treatment of this condition is not entirely clear. Alternative or supplementary pharmacological treatments now exist only on an experimental basis.High-quality, prospective, randomized trials that would enable a definitive judgment of the efficacy of HBOT are currently lacking.