In the Dominican Republic, the phenomenon of children who were raised female appearing to swap sexes at puberty is so common it even has a name. Guevedoces roughly translates to "penis [or "balls"] at 12," and it’s the result of a rare enzyme deficiency that delays crucial steps of male sexual development until puberty.

When guevedoces are born, they appear to have external female genitalia even though their genes and internal reproductive organs are male. Parents assume their children are girls and raise them as such. But when these children begin producing large amounts of testosterone at puberty, their testes descend and they grow a penis—in addition to all the other changes that come along with male adolescence.

Sexual development normally begins in the womb, and the same is true for guevedoces. Whether the fetus has one X chromosome or two, for the first several weeks of development its genes follow the same blueprint for both sexes. Then, sometime around the eight-week mark, the sex chromosomes get to work. For males, the undeveloped gonads become testicles and they start to release male hormones, including testosterone. In a structure called the tubercle, an enzyme called 5-alpha-reductase converts the testosterone to a stronger hormone called dihydrotestosterone (DHT), which is necessary to develop external genitalia. It's this hormone that turns the tubercle into a penis; without it, it develops into a clitoris.

The rare enzyme deficiency found in guevedoces leaves them unable to develop external male genitalia in the womb. They still produce plenty of testosterone, which triggers the development of internal structures like the epididymis and vas deferens, but the lack of DHT makes the babies appear female at birth. It’s not until the second surge of testosterone these children receive at puberty that they grow testes and a penis.

The condition is thought to be genetic, tracing back to the female founder of a small village in the Dominican Republic's mountainous hinterland. Outside of the nation, it’s incredibly rare.

For some guevedoces, being raised as female wasn’t an easy experience. “I never liked to dress as a girl, and when they bought me toys for girls, I never bothered playing with them,” Johnny, who had grown up as Felicita, told BBC Two, which features these kids in the second episode of the series Countdown to Life: The Extraordinary Making of You. “When I saw a group of boys, I would stop to play ball with them.” When Johnny, now 24, began to undergo physical changes, he was taunted at school and called nasty names by his classmates. He’s had a number of short-term girlfriends since going through puberty and dreams of one day getting married and starting a family. Another child named Carla began the process of transitioning to Carlos at age 9; he can be seen receiving a smile-inducing haircut in the photo above.

Most people with this condition live out their adult lives as men, but some choose to undergo surgery and remain female. The discovery of this disorder in the 1970s led to the development of a best-selling drug called finasteride, which is commonly prescribed to treat benign enlargement of the prostate and male pattern baldness. (You may know it by the brand name Propecia.) The drug mimics the enzyme deficiency by blocking the action of 5-alpha-reductase.

You can learn more about this rare condition and the people who have it on the BBC Two series Countdown to Life: The Extraordinary Making of You.

[h/t: BBC]