Objective: To report two rare cases of persistent müllerian duct syndrome.
Methods/results: Two patients with normal chromosome studies (46, XY) are presented. The radiological evaluation showed structures compatible with those of the müllerian duct. These structures were completely excised with excellent results. The histological findings confirmed the clinical diagnosis.
Conclusions: Persistent müllerian duct syndrome is a rare form of male pseudohermaphroditism that presents difficulties in making the differential diagnosis from other conditions with a different etiopathogenesis and that are not completely defined.