Abstract
Fraser syndrome is a rare disorder with autosomal recessive inheritance having a wide spectrum of phenotypic appearances. A fourteen-year-old female presented to us with a chief complaint of acute retention of urine without any cyclical abdominal pain with syndromic appearance. She had partial vaginal agenesis which was treated by successful reconstructive surgery by lotus petal flap technique followed by recanalisation. Objective of this report is to highlight the rare disease of Fraser syndrome along with successful rare surgical management.