[5][6] Typical features include undescended testes (cryptorchidism) and the presence of a small, underdeveloped uterus in an XY infant or adult.

PMDS Type I is also referred to as hernia uteri inguinalis, which exhibits one descended testis that has also pulled the fallopian tube, and sometimes uterus, through the inguinal canal.

[11] However, for females who originally do not produce AMH proteins during foetal development, the Müllerian duct eventually becomes the uterus and fallopian tubes as normal.

[10] The male individuals inherit mutated copies of the X chromosomes from the maternal and paternal genes, implying the parents are carriers and do not show symptoms.

In PMDS, an abnormality in the anti-Müllerian hormone signaling pathway causes the in-males-redundant Müllerian duct to persist and give rise to variously developed female reproductive organs.

At approximately the end of the 7th and the beginning of the 8th week of gestation, the Sertoli cell's secretion of AMH occurs, causing male sex differentiation during fetal development.

[11] The AMH molecules bind to AMHRII (anti-Müllerian hormone receptor type II) regressing the Müllerian duct.

The Leydig cells secrete testosterone to aid the male differentiation process by inducing structures such as the epididymis, vas deferens and seminal vesicles.

Laparotomy properly positions the testes within the scrotum (orchidopexy) and removes Müllerian structures, the uterus, and fallopian tubes.

During this surgery, the uterus is usually removed and attempts made to dissect away Müllerian tissue from the vas deferens and epididymis to improve the chance of fertility.

If the person has male gender identity and the testes cannot be retrieved, testosterone replacement will usually be necessary at puberty should the affected individual choose to pursue medical attention.

Recently, laparoscopic hysterectomy is offered to patients as a solution to both improve the chances of fertility and to prevent the occurrence of neoplastic tissue formation.

Upon pathohistological observation, the endometrial tissues appear atrophied, and the fallopian tubes have begun to congest showing signs of fibrosis.

A case reported in 2013, involves a 50-year-old male with a history of low testosterone levels, high cholesterol and the congenital absence of his right testis.

[13] During the operation, the surgeons found malignant degeneration of the Müllerian remnants which occurs if PMDS is unnoticed for a long period of time.

[13] Overlooking the symptoms of PDMS can cause permanent negative effects such as infertility and future malignancies, as shown by this male patient.