Pseudoaneurysm of the mitral‐aortic intervalvular fibrosa (P‐MAIVF) continues to be a rare diagnosis in children. Etiology, presentation, and management strategies are considerably different from adults. We report a fatal case of P‐MAIVF with classical transthoracic and transesophageal echocardiographic findings complemented by CT imaging. The natural course of uncomplicated/asymptomatic P‐MAIVF is largely unknown since most patients are offered surgery. We present an extensive literature review of pediatric P‐MAIVF and highlight important differences from the adult form of this disease.