We describe deep venous thrombosis and a circulating anticoagulant in a male adolescent with systemic lupus erythematosus (SLE). The association of deep vein thrombosis with SLE in a pediatric patient has not, to our knowledge, been previously reported. The circulating anticoagulant was characterized as a lupus-type inhibitor. This was demonstrated by an abnormal partial thromboplastin time (PTT), the failure of the PTT to correct with the addition of an equal amount of normal plasma, and a positive tissue thromboplastin inhibitor test. Physicians should be aware that a circulating anticoagulant can be associated with SLE and that there may be a paradoxically increased incidence of thromboembolic phenomena in patients with this abnormality.