Type III longitudinal deficiency of tibia according to Kalamchi and Dawe denotes the presence of distal hypoplasia of the tibia with diastasis. We report a case of type III longitudinal deficiency of the tibia in a female patient who later underwent reconstructive surgery. The first reconstruction of the leg was done when child turned 4 months of age. Surgical procedures included foot reconstruction and ankle stabilization with twice lengthening by the Ilizarov method (14 cm in total). During the follow-up, both the tibia and fibula of the affected leg showed the same lengthening and regression due to preserved distal growth zone cartilage. After surgical correction, the acetabulum was satisfactorily configured with an acetabular angle of 23 degrees. Explanation for surgical success was that osteotomy and distraction were done in the proximal part of the crural region where the growth potential was better. The tibia remained lean and hypoplastic while the fibula was incrassated. The function in the area of the knee joint was preserved, while the distal part of the leg served as good stand on. When the child was 18 years old, on check-up, the acetabular angle was 23 degrees while the Wiberg angle was 24 degrees.