Introduction: Tibia hemimelia is a longitudinal deficiency of tibia bone which is manifested at birth. Limb shortening, severe foot deformity, and knee flexion contracture are presented in this disease. It is usually treated during the early years of life. The goal of treatment is to achieve stable and equal length in both legs.

Case report: The mentioned case is a 20 year old man with type IA Tibia hemimelia. The interesting point in this case is the tolerance of severe right lower limb deformity, shortening with an extra thumb and toe on the same side till the age of 20 years.

Conclusion: It is best to treat this condition in the first few years of life and as minimum as possible operations should be imposed to the patient.