This case report represents a rare case of 14-year-old female who diagnosed with hypocompementemic urticarial vasculitis syndrome that presents with glomerulonephritis, diffuse alveolar hemorrhage, and acute disseminated encephalomyelitis. The progression of the symptoms explained in the text below in which the final diagnosis was reached after a challenging approach. Patient was managed properly and followed up after treating with rituximab, although she represents no sign of the disease after a total of two cycles.