Introduction: Kawasaki disease is an acute self-limiting febrile vasculitis of unknown aetiology which is commonly seen in children six months to five years of age. It is characterized by inflammation of medium sized arteries with a predilection for coronary arteries and the mostdevastating complication is, coronary artery dilatation and aneurysm formation Case presentation: A three-month-old female infant previously thriving well presenting with ahigh-grade fever for over five days, a generalized erythematous macular rash, subtle oral mucosal redness and dry , cracked lips. No strawberry tongue observed. Other major criteria like lymphadenopathy , conjunctival suffusion or extremity changes were not observed. Infant appeared acutely ill. Biochemical findings were not strongly supportive of the diagnosis, with only a marginally elevated CRP of 40? mg/Land hemoglobin of 10.4? g/dL,providing partial evidence suggestive of an atypical presentation. However, the 2D Echocardiogram demonstrated marginal dilatation (Z score – 2.0-2.5) of LMCA, LAD and RCA consistent with early coronary involvement. These findings were suggestive of atypical Kawasaki and treatment started with IV immunoglobulin and a course of oral aspirin and oral steroids. With treatment infant recovered completely along with normalization of inflammatory markers and 2D echo at four weeks showed dilated RCA only . Conclusion: Kawasaki disease in children less than six months is rare, and the clinical presentations can be subtle and nonspecific. Therefore, is a diagnostic challenge and need to maintain high index of suspicion in prolonged febrile illness with no clear source of infection. Laboratory and echocardiographic evaluation are essential for diagnosis.