Human Leukocyte Antigen (HLA) B27 associated arthritis is a distinct subtype of chronic arthritis characterized by genetic predisposition, arthritis with axial involvement, enthesitis and anterior uveitis. Extra–articular manifestations are not uncommon in this subgroup of patients with JIA . We report a 12–year–old boy who presented with bilateral knee and right hip joint arthritis for 3 months duration and HLA B27 positivity. On examination he had arthritis of bilateral knee joints and peripheral signs of aortic regurgitation. Two dimensional echocardiography revealed moderate aortic regurgitation. Although, cardiac abnormalities like valvular insufficiency, valvular incompetence and conduction defects are commonly reported in adults with prolonged disease course, but these are rarely seen in children and is exceptional at such a young age and so early in disease course. So baseline and regular CVS evaluation including echocardiography needs to be carried out in all young patients with HLA B27 associated arthritis.