Congenital lymphedema of the external genitalia is a rare, disfiguring disorder. We describe here a case of a 3-year-old male with primary foreskin lymphedema persisting since birth. A compact, heterogenous swelling of the foreskin's distal third was observed, inhibiting preputial retraction (phimosis). Right lower extremity lymphedema was also observed in this case, while no further abnormalities were found. Surgery was performed, maintaining the foreskin, producing an excellent result with no recurrence at 10-month follow up.
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