Persistent müllerian duct syndrome (PMDS) is a rare form of male pseudohermaphroditism. We present 5 cases with PMDS (2 cases associated with testicular malignancy) and discuss the diagnosis and management. Management strategies of PMDS have changed. Whereas in the past, removal of the müllerian remnants was targeted together with orchidopexy or -ectomy, this is no longer recommended. However, testicles that cannot be descended at an early stage are at a high risk of malignancy and should, therefore, be removed. If this is necessary on both sides, there is the additional problem of lifelong testosterone substitution which requires efficient patient monitoring and good patient compliance. In cases where this cannot be achieved, compromises, such as temporarily delayed orchidectomy, may be considered.
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