Background: Mayer-Rokitansky-Küster-Hauser syndrome (MRKH) occurs in 1:5,000 births. It is a congenital disorder in the formation of the Müllerian ducts and therefore presents a primary amenorrhea, due to absence of vagina and uterus, with functional ovaries. Secondary sexual characteristics develop, having a normal female karyotype (46, XX). Women with the condition suffer from uterine factor infertility. The uterine factor is part of multifactorial infertility in 50% of cases; but, considered as the only responsible factor, its incidence is between 5-12%Objective: Report pediatric patient MRKH anomaly with description of management options.Case presentation: A 15-year-old female, Tanner IV breast development, absent vagina and with amenorrhea. Estradiol 56 pg/ml, FSH 5.7 mIU/ml, LH 10 mIU/ml, karyotype 46,XX. Pelvic ultrasound with ovaries present, uterine remains. Simple pelvic magnetic resonance: uterine remains, ovaries present, vaginal agenesis. Renal ultrasound and chest X-ray normal. Vaginoplasty surgery, removal of uterine remains. Current sexually active life is normal.Conclusion: The diagnosis of MRKH syndrome type 1 often occurs in late adolescence, when a young woman consults because she has not started her menstrual period. A diagnosis of MRKH Type 2 may be triggered by an absent period or a wide range of other symptoms or organ-related abnormalities, sometimes at an earlier age. There are surgical and non-surgical options for treating MRKH. Depending on your individual condition, you may benefit. These patients require comprehensive, interdisciplinary management to promote their full rehabilitation.