Extrapulmonary comorbidities are common and significant in chronic obstructive pulmonary disease (COPD), often contributing to symptoms, exacerbations, hospital admissions and mortality. Cardiovascular, musculoskeletal and psychological conditions are among the most prevalent and important of these. In particular, ischemic heart disease is a leading cause of death in the COPD population as a whole. Here, we provide a state-of-the-art summary of key comorbidities observed in COPD patients in terms of their prevalence, impact, pathophysiology and prognosis. In addition, we review clinical, diagnostic and management strategies that may differ in COPD patients from the rest of the population.