Kawasaki disease (KD), also known as Kawasaki syndrome, is an acute febrile illness of unknown cause that primarily affects children younger than 5 years of age. The present study was conducted to study the clinical profile and outcome and risk factors of Kawasaki disease in children. This observational prospective study was conducted in cardiology ward with a diagnosis of Kawasaki disease from 1st June 2020 to 31 July 2021 in Bangabandhu Sheikh Mujib Medical University Hospital Dhaka, Bangladesh. Forty five (45) children aged between 3 months to 10 years were studied. Kawasaki disease is more prevalent among male child (73.33%). Most commonly encountered risk factor for cardiovascular involvement especially coronary dilatation or aneurysmal change is more observed in younger age of presentation. Fever, rash, conjunctivitis, erythema and edema of limbs, periungual skin desquamations are the presenting features. Cardiovascular involvement of left main coronary artery dilation is the most frequently observed aneurismal change observed among study population which was evident in 68% of the children having coronary involvement. All the study patient received high dose aspirin (100%) and 93% received IVIG of whom 6.66% had IVIG- resistant Kawasaki disease. Commonly occurring complication is uveitis (17.77%). Among the total 45 study population 1 patient expired (2.22%).Clinicians should have a high index of suspicion of Kawasaki disease in persistently febrile patients and once clinically diagnosed, echocardiography should be done and IVIG therapy along with aspirin should be started, specially in infants (<6 months) as they have the higher tendency to develop coronary aneurysm.