Background: Neisseria meningitidis is a gram-negative bacterium with the potential to cause invasive disease. Invasive meningococcal disease (IMD) can be fatal if delay to antibiotic therapy. There are six serogroups, which are capable of causing invasive disease in humans; A, B, C, W, X and Y. Since 2015, serogroup W and serogroup Y account for the majority of IMD cases reported in Sweden. Aim: To investigate the clinical presentations of IMD caused by Neisseria meningitidis serogroup W and Y. Method: Two databases, PubMed and Cochrane, were used to find articles that described the clinical picture of IMD. Articles with description of clinical features of the studied serogroups and with eight cases or more in every study were included. In addition, only original articles were included. Results: A total of 633 articles were found and 11 fulfilled all the inclusion criteria. Five out of seven articles found meningococcemia as the predominating presentation of serogroup W IMD. Two out of the four articles that studied serogroup Y IMD found meningitis at a higher number. Conclusion: The results of this systematic review suggest meningococcemia as a relatively common presentation of serogroup W IMD while meningitis and pneumonia might occur more frequently in serogroup Y IMD. However, these results should be interpreted carefully because the included articles were mostly retrospective studies and future prospective studies are needed to better identify clinical presentations of serogroup W and Y IMD.