Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disease. Among the cutaneous manifestations of SLE, digital gangrene is considered to be very rare. This complication, which may lead to severe ischemic necrosis and amputation, is suggested to be the result of poor perfusion that is usually caused by vasculitis, vasospasm, thromboembolism, or atherosclerosis. Digital gangrene is seen mostly at a late stage of the disease proposing that a long history of SLE is a considered risk factor. Only 0.2% of patients with SLE presented initially as digital necrosis. This is a case report of a 20-year-old Saudi female who presented to the emergency room primarily with acute painful localized dry digital gangrene associated with bilateral lower limbs petechial rash. Her medical history was not suggestive of autoimmune diseases. Serology was positive for SLE. A diagnosis of SLE, lupus nephritis, and vasculitis has been established clinically and serologically. The patient adequately responded to rituximab and steroids as a medical therapy. To our knowledge, cases of acute peripheral gangrene as the initial and only presentation of SLE have rarely been documented in Emergency Medicine.