Two newborn boys aged 2 and 3 months with unilateral inguinal hernia and a contralateral impalpable, non-scrotal testis, and a third boy aged 2.5 years with an impalpable non-scrotal testis were found to have transverse testicular ectopia. This is an uncommon abnormality in which both gonads migrate toward the same hemiscrotum. We illustrate that unilateral cryptorchidism and a contralateral inguinal hernia may indicate the presence of a rare type of male pseudohermaphroditism: persistent müllerian duct syndrome (PMDS). This syndrome is characterized by the presence of a uterus and fallopian tubes associated with abdominal testes and frequently inguinal hernia in a phenotypically and genotypically normal male. This syndrome is often discovered during repair of inguinal hernia or non-descended testes (cryptorchidism). Pre-operative ultrasonography in children with impalpable non-scrotal testis and a contralateral inguinal hernia (patent processus vaginalis) may enable an early diagnosis of transverse testicular ectopia and proper surgical planning. Surgical orchidopexy was carried out and in the first two patients resection of the müllerian duct remnant (utriculus masculinus).