Hyperketonemia (HYK) is one of the most frequent and costly metabolic disorders in high-producing dairy cows and its diagnosis is based on β-hydroxybutyrate (BHB) concentration in blood. In the last 10 years, the number of papers that have dealt with the impact of elevated BHB levels in dairy cattle has increased. Therefore, this paper reviewed the recent literature on BHB concentration in blood and milk, and its relationships with dairy cow health and performance, and farm profitability. Most studies applied the threshold of 1.2 mmol/l of BHB concentration in blood to indicate HYK; several authors considered BHB concentrations between 1.2 and 2.9 mmol/l as subclinical ketosis, and values ⩾3.0 mmol/l as clinical ketosis. Results on HYK frequency (prevalence and incidence) and cow performance varied according to parity and days in milk, being greater in multiparous than in primiparous cows, and in the first 2 weeks of lactation than in later stages. Hyperketonemia has been associated with greater milk fat content, fat-to-protein ratio and energy-corrected milk, and lower protein and urea nitrogen in milk. The relationships with milk yield and somatic cell count are still controversial. In general, HYK impairs health of dairy cows by increasing the risk of the onset of other early lactation diseases, and it negatively affects reproductive performance. The economic cost of HYK is mainly due to impaired reproductive performance and milk loss. From a genetic point of view, results from the literature suggested the feasibility of selecting cows with low susceptibility to HYK. The present review highlights that milk is the most promising matrix to identify HYK, because it is easy to sample and allows a complete screening of the herd through BHB concentration predicted using mid-IR spectroscopy during routine milk recording. Further research is needed to validate accurate and convenient methods to discriminate between cows in risk of HYK and healthy animals in field conditions and to support farmers to achieve an early detection and minimise the economic losses.