Background: Kawasaki disease is an acute systemic vasculitis that predominantly affects young children and is rarely seen in adults. In the recent years, there have been a few cases in the literature reporting similar clinical picture in adults, especially in those with concurrent HIV, which lead us to present our findings and explore the illness further. Case report: A 20-year-old African American female with congenital human immunodeficiency virus (HIV) infection, presented to the ER with complaints of chronic fatigue, fever (Tmax 103 8F), arthralgia and sore throat since 2–3 weeks. She also had generalized pain, swelling and erythema of both hands and feet as well as conjunctival erythema with watery discharge since the same duration. An extensive infectious disease and rheumatologic work up in the hospital was negative. She had two prior admissions for similar complaints in less than a year with minimal response to NSAID and prednisone. The patient was started on a 5-day course of IV Immunoglobulin and Aspirin for suspected Kawasaki-like disease (KLD). She responded well and her symptoms resolved in a few days, strengthening our diagnosis. Conclusion: It is a challenging task to diagnose Kawasaki-like disease in HIV infected adults due to a high likelihood of opportunistic infections or possible hypersensitivity to any one of the plethora of prescribed medications. A high suspicion of KLD is advised in HIV infected adults with severe immunosuppression and unexplained fever lasting > 5 days to ensure timely diagnosis and treatment.