Mayer-Rokitansky-Küster-Hauser sundrome belongs to class I Müllerian duct anomalies (uterine agenesis). In this case; the uterus is missing and upper segment of the vagina with the presence of both ovaries, the right ovary is extra-pelvic at the inguinal region. This is associated with right renal agenesis. There is an uncommon association with renal agenesis and extrapelvic ovary that is demonstrated in this case. Primary amenorrhea is the most common presentation as the presence of functioning ovaries and normal hormonal levels preserve the external genital development and puberty.