Despite the development of pharmacological therapies that are effective in slowing the progression of idiopathic pulmonary fibrosis (IPF), it remains a debilitating and lethal condition. In addition to the adverse effects caused by pulmonary fibrosis, most patients with IPF have associated comorbid conditions, which might negatively affect functional status, quality of life, and survival. Comorbid conditions can be pulmonary or extrapulmonary. Pulmonary comorbidities include pulmonary hypertension, emphysema, and lung cancer, while non-pulmonary conditions include venous thromboembolism, coronary artery disease, congestive heart failure, sleep-disordered breathing, gastro-oesophageal reflux disease, and anxiety or depression. Although some of these comorbid conditions share risk factors with IPF, the likelihood for their presence or development in patients with IPF is still greater than expected. This might indicate that IPF fosters an environment for the development or perpetuation of comorbid conditions, or alternatively that they share causative factors. Optimal management of IPF therefore requires a comprehensive approach, which includes the identification and treatment of comorbid conditions to optimise patient outcomes.