Hydrometrocolpos (HMC) is the distension of the vagina and uterus by accumulated mucus secreted by the uterine and cervical glands stimulated in utero by maternal estrogen (secretary HMC). The other mechanism of HMC is the accumulation of urine in the presence of a vaginal obstruction (urinary HMC). Congenital vaginal obstruction causing HMC is due to imperforate hymen, transverse vaginal septum, vaginal atresia, or persistence of urogenital sinus with complete distal vaginal obstruction. Imperforate hymen usually remains asymptomatic until puberty. Rarely, symptoms of imperforate hymen manifest antenatally as well as in the neonatal period, requiring surgical correction for life threatening consequences. We report a case with marked congenital hydrometrocolpos and imperforate hymen at a four-day-old female newborn.