Background: Immunoglobulin A vasculitis (IgAV) is one of the most common vasculitis in children. It is generally a self-limiting disease. Due to its systemic nature, a variety of symptoms in different organs can be observed. We report a case of IgAV characterized by several complications to improve clinicians’ understanding of the disease. Case presentation: A 4-year-old boy was admitted to a local hospital because of abdominal pain and skin rash. The skin biopsy showed leukocytoclastic vasculitis with IgA deposition, consistented with a diagnosis of IgAV. He developed clinical signs of intussusception and laparotomy was undertaken. He continued to have intermittent abdominal pain and edema in the four limbs with oliguria. Elevated pancreatic enzymes and swelling of the pancreas on abdominal ultrasound suggested a combination of pancreatitis in the child. The child subsequently developed headache, dizziness and convulsions, and head MRI showed a high signal on the left side of the cortex and subcortical white matter, and he was considered to have developed cerebral vasculitis.He underwent bronchoscopy because of respiratory distress, which which confirmed the presence of pulmonary hemorrhage. Combined pulmonary infections added to the severity and complexity of his condition. He received two courses of methylprednisolone pulse therapy combined with IVIG and aggressive anti-infective therapy, but his condition eventually deteriorated and he died. Conclusions: IgAV can involve multiple systems and various complications. There is no definitive evidence to support a single drug or multi- immunosuppressive regimen. IgAV usually runs a benign course,however, the severe cases are critical, with a high mortality rate.