Tibial hemimelia is the rarest form of congenital longitudinal deficiency of the tibia with an incidence of 1:1,000,000 live births. It frequently presents with associated anomalies like herniae, gonadal malformations, hypospadias, cleft palate, imperforate anus and 1 congenital heart disease. There are also recognised syndromic presentations . However, a complex presentation in which the knee is f ully flexed and the leg is embedded into the posterior aspect of the ipsilateral thigh and labia majora as in the case presented has not been reported in the literature.

Keywords: Tibia hemimelia , complex presentation.