True saccular aneurysm is exceptional in coarctation of aorta in children. A 6-year-old girl with headache and systemic arterial hypertension referred to our center for cardiovascular evaluation. Physical examination revealed high blood pressure and weak lower extremity pulses. Two-dimensional and Doppler echocardiography and angiography demonstrated a saccular aneurysm of the descending aorta, 5×4 cm in size, associated with aortic coarctation. Aneurysmectomy was performed without cardiopulmonary bypass. The section of the aorta containing the coarctation and the aneurysm was resected and replaced with a 15-mm woven polyester graft. Histological examination revealed a thin walled aneurysm with hyalinosis changes and decreased elastic fibers.One-year echocardiographic follow-up revealed no gradient across of Dacron tube and hypertension decreased to normal level.