Anasarca as the presenting manifestation of juvenile dermatomyositis (JDMS) is extremely rare. We report a case of a 4-year-old boy who was initially managed for nephrotic syndrome in view of anasarca and mild hypoalbuminemia. Later, at presentation to our institute, a diagnosis of severe edematous JDMS was made in view of associated profound muscle weakness and characteristic skin changes. The child responded to aggressive immunosuppressive therapy. On further evaluation, he had evidence of acute parvovirus B19 infection. Our case illustrates anasarca as an uncommon severe manifestation of JDMS and the possible role of parvovirus B19 in the onset of this autoimmune disorder.