Nontraumatic, spontaneous parenchymal kidney rupture is a rare clinical entity that may cause extensive hemorrhage, hypovolemic shock, and death. Spontaneous nontraumatic kidney rupture is extremely rare in systemic lupus erythematosus (SLE) patients. Because of the high morbidity and mortality rates, an immediate establishment of the diagnosis and treatment are necessary. We present the case of a 30-year-old female with spontaneous parenchymal rupture of the right kidney who had renal failure due to SLE and presented with atraumatic sudden right flank pain during hemodialysis treatment. To our knowledge, this case is the second report of SLE manifesting as spontaneous kidney rupture in the literature.