Systemic lupus erythematosus (SLE) is an autoimmune disease that can involve any organ system, exhibiting great diversity in presentation. Cardiac tamponade as the initial presentation of childhood onset SLE (cSLE) is rare. We report the case of a 10 year old Afro-Caribbean female who presented with complaints of chest pain, shortness of breath and fever over 4 days. Clinical examination strongly suggested cardiac tamponade which was confirmed by investigations and treated with pericardiocentesis. After a thorough investigation, the underlying diagnosis of SLE was confirmed using the Systemic Lupus International Collaborating Clinics (SLICC) criteria and high dose corticosteroid therapy initiated. A review of recent studies shows that common initial presentations of cSLE include constitutional symptoms, renal disease, musculoskeletal and cutaneous involvement. In presenting this case and reviewing the literature we emphasize the importance of cSLE as a differential diagnosis when presented with pericarditis in the presence or absence of cardiac tamponade. In these patients early diagnosis and treatment is desired and in this regard we also discuss the sensitivity of the SLICC criteria in cSLE.