Primary amenorrhea is a challenging entity as it affects the reproductive outcome. Primary amenorrhea is an absence of secondary sexual characters until 14 years of age or absence of menstruation with secondary sexual characters until 16 years of age.

The aim of this article is to study the frequency, etiologic causes, presentation, diagnosis, and optimal mode of management of primary amenorrhea.

All girls who had not attained menarche until 16 years of age in the absence of secondary sexual characters and until 14 years, if no secondary sexual characters, and were willing for follow-up were subjected to investigations and were treated as per the etiology.

Mayer–Rokitansky–Küster–Hauser (MRKH) syndrome was the most common cause of primary amenorrhea (42.85%) followed by imperforate hymen (28.57%) and transverse vaginal septum (21.42%), one each of low, mid, and high varieties. There was one case of androgen insensitivity syndrome.

Establishing correct diagnosis is essential for planning treatment and management strategies in primary amenorrhea as treatment goals are preservation of fertility and progression of normal development.

Bhalerao AV, Garg R. Müllerian Anomalies: A Cause for Primary Amenorrhea. J South Asian Feder Obst Gynae 2017;9(3):221-224.