The number of times a scientific article has been cited reflects its impact on a specific field. Highly cited articles are known as citation classics. Here, we aim to characterize the most frequently cited articles in chronic granulomatous disease (CGD). We searched the ISI Web of Science in Aug 2016 for articles that were cited 100 times or more and evaluated them for several characteristics. The most frequently cited article received 955 citations. The citations mean was 221.6 citations (SD = 162.7). The most recent article was published in 2011. The articles were published in 36 journals, led by The New England Journal of Medicine. Overall authors came from 17 countries, with the United States of America (USA) contributing to 91 (80%) articles. The National Institute of Health was the most common institution of origin for the corresponding author. The most common type of articles was basic science with 77 (67%) articles. These results provide some insights into the most cited research articles in CGD since its first description 60 years ago.