ABSTRACT
In children, the lymphedema often results from a primitive abnormality of lymphatic vessels. Through four observations depicted in the pediatric department of Sfax, we try to show the clinical and etiological polymorphism of this disease and to underline the difficulties of the coverage especially at the child. 1: a girl hospitalized at birth for Bonnevie-Ullrich's syndrome associating an innate lymphedema and Turner's syndrome. A boy hospitalized at the age of 13 months for congenital unilateral lymphedema with penio-scrotal atteinte, associated to urinary malformation [not obstructive pyelo-ureteral connective syndrome]. A 7 years old boy hospitalized for familial primitive premature bilateral lymphedema type Meige. A 13 years old boy, hospitalized for recidivated erysipele. The diagnosis of sporadic primitive premature unilateral lymphedema associated to venous malformation was retained