ABSTRACT Ovarian cysts are common causes for gynecological surgery. Juvenile hypothyroidism, multiple large ovarian cysts causing acute abdomen and primary amenorrhea are infrequently reported and not widely recognized. We report a case of a 21-year-old lady who presented with juvenile hypothyroidism, primary amenorrhea, multiple cystic ovary and raised thyroid stimulating hormone (TSH), follicle stimulating hormone (FSH) and estradiol levels. After thyroid replacement, regression of TSH, FSH levels and cyst size were noted. Laparotomy done in view of torsion of ovary, histopathology (HPE) revealed multicystic ovary. This case was reported for its unusual presentation of hypothyroidism leading to torsion ovary. How to cite this article Pushpalatha B, Potluri R, Lakshmi V, Seshadri KG, Sireesha PSNRS. Acute Abdomen, Primary Amenorrhea, Juvenile Hypothyroidism: A Rare Combination. J South Asian Feder Obst Gynae 2015;7(3):236-239.