Background: Mayer-Rokitansky-Kuster-Hauser syndrome (MRKHS) is characterized by absent or rudimentary uterus and the upper part of the vagina, is the second most common cause of primary amenorrhea after gonadal dysgenesis. Content: The presentation reviews the role of different imaging modalities in the diagnosis of MRKHS. An extremely rare case of MRKHS associated with a left inguinal ovary in a young woman is also discussed. A 21-year-old woman presented with primary amenorrhea. The patient had a female phenotype, normal stature, and normal secondary sex characteristics. Physical examination showed normal hymenal fringe and a blind pouch of vagina. Pelvic MRI performed with a 3.0T (Siemens Skyra) scanner (coronal plane T2WI with TR-4032ms, TE-71ms; transverse plane T2WI with TR-11140 ms, TE- 102ms and sagital plane T1WI with TR-879ms, TE-11msș slice thickness 4-6mm) revealed normal bilateral position of the kidneys, absent uterus, cervix, and proximal (upper) vagina. Fibrofatty tissue between the bladder and the rectum in the expected location of the vagina was also noted. The right ovary appeared normally sited with follicles in various stages of maturity. The left ovary, however, had atypical location in the left inguinal canal, with a relatively normal structure and small follicles. The diagnosis of MRKHS was confirmed. Conclusions: MRKH syndrome is a congenital disorder of the female genital tract caused by the maldevelopment of the Müllerian duct. The incidence of ectopic ovary in MRKHS is ranging from 15% to 42%. The ovarian position serves a pivotal role in the strategy of gestational surrogacy. MRI is a useful and noninvasive imaging method in the diagnosis and evaluation of patients with MRKHS.