Roentgenogram of the chest and abdomen were suggestive of right-sided pleural effusion and ascites. Chromo-somal study was normal. He was given supportive and symptomatic treatment. Congenital lymphedema is a rare entity. Primary lymphatic dysplasia is a term used to describe congenital abnormality of lymphatics which consist of lymphedema, chylous ascites and chylothorax in children.
Genital involvement is an uncommon feature.
Diag-nosis is mainly clinical. Investigations like lymphangiograms, venograms and biopsies, CT scan, pelvic or abdominal USG usually add little to the diagnosis in children. The management of such cases is ill defined. Medical management used comprises steroids, diuretics and diet while surgical management includes repeated aspirations and various operative procedures. Showing lymphedema of right side of body with ascites and genital involvement.