The International Classification of Diseases (ICD), which is used to group and report health conditions and factors, provides a basis for healthcare statistics. The 11th revision of the ICD (ICD-11) released by the World Health Organization provides stakeholders with novel perspectives on solving the complexity of critical problems in medical informatics. This study conducts a bibliometric analysis of research published over the period of 1989–2018 to examine the development of ICD-related research and its trends. First, over 4000 ICD-related papers spanning the 30-year period are retrieved from the Web of Science database. Then, based on the meta data of the selected papers, time trend analysis is performed to examine the development of different ICD revisions. Finally, the keywords and topics of these papers are analyzed and visualized using VOSViewer and CiteSpace. Our findings indicate that ICD-11-related research has grown rapidly in recent years compared with studies on ICD-9 and ICD-10. Moreover, the most popular research directions of ICD-11 include the topics psychiatry, psychology, information science, library science, and behavioral science. In terms of perspectives, information system-related research is more common than big data- and knowledge discovery-related work. However, the popularity of big data- and knowledge discovery-related developments has grown in recent years. The use of ICD-11 facilitates the development of medical informatics from the perspectives of information systems, big data, and knowledge discovery.