Lupus anticoagulant-hypoprothrombinemia syndrome (LA-HPS) is a rare disorder, and appears mostly in children. The primary concern is its potential development into systemic lupus erythematosus (SLE). A 5-year-old patient was hospitalized with multiple purpuric lesions. A markedly prolonged prothrombin time and activated partial thromboplastin time were observed and were not corrected after mixing with normal plasma. Decreased factor II activity was consistent with LA-HPS. Identifying risk factors that play an important role in the development of SLE in patients with LA-HPS is of importance. Based on the case described here, anti-double stranded (ds) DNA antibody and the Sapporo criteria for antiphospholipid syndrome are related to subsequent SLE development, whereas there is no correlation with the results of the lupus anticoagulant (LA) test. We recommend an early and serial examination of anti-ds DNA antibody and full evaluation of Sapporo criteria for the screening of patients with LA-HPS who may progress to SLE.