Systemic lupus erythematosus (SLE) is a serious chronic autoimmune disease with intense inflammatory response and damage in many target organs including joints, skin, kidneys, heart and nervous system. Cardiac tamponade is extremely rare as a cardinal presentation of SLE in children with only a few cases reported in the literature. We report two cases of a 9-year-old boy and an 11-year-old girl presenting with acute cardiac tamponade and later recognition of elevated anti-double-stranded DNA (anti-dsDNA) titre. We also present a literature review about similar cases in children and we stress on the importance of screening all cases of acute cardiac tamponade in children with antinuclear and anti-dsDNA antibodies to avoid any delay in SLE diagnosis and treatment.