We describe a 10-month-old infant with PMDS successfully managed by the laparoscopic approach. At the age of 1.5 months, the patient presented with a left inguinal hernia and bilateral nonpalpable gonads in another center and underwent left inguinal exploration. The uterus and a gonadlike structure along with the hernia sac were found in the inguinal canal. Left inguinal herniotomy was performed after reduction of the uterus and gonadlike structure. No gonadal biopsy was obtained. The patient was further investigated in the same center. His karyotype was 46,XY. Magnetic resonance imaging of the abdomen and pelvis revealed a uterinelike structure posterior to the urinary bladder, but neither testis nor ovaries were visualized. At 10 months of age, he was referred to our department for furth...