Antiglomerular basement membrane antibody disease (aGD) remains a very uncommon entity in the pediatric population, characterized by pulmonary hemorrhage, glomerulonephritis, and antibodies against the glomerular basement membrane. We herein describe the case of a 14-year-old girl with primary pulmonary symptoms diagnosed with aGD. Furthermore, we review 23 cases described in English literature over a 25-year period. Our case is the fourth child that presented with only pulmonary symptoms and no or minimal renal involvement. Based on the 23 cases, we present data on demographics and clinical symptoms, diagnostic tools, and prognosis. aGD presents in children with a male/female ratio of 1:2. Most children (91 %) survive, but in many cases renal disease progresses to end stage, requiring maintenance therapy on dialysis and some undergo kidney transplantation. However, no case was identified with signs of relapse after remission. aGD should be considered in the differential diagnosis of diffuse lung hemorrhage despite the lack of renal abnormalities. Antiglomerular basement membrane antibody testing can be of great value in confirming the diagnosis.