In skeletal dysplasia, various types or deformities may develop around the joints due to metaphyseal and epiphyseal growth disturbances. One of the most common location of these growth disturbances and deformities is the hip joint. The delay in ossification of the proximal femur epiphysis and growth plate abnormalities can cause femoral head flattening and violate the relationship between the femoral neck and the shaft. Femoral head flattening may be followed by lateral subluxation and different types of proximal femur deformities. The two main deformities emerging in the proximal femur are the coxa vara and the coxa valga in which the femoral neck and shaft angle is decreased and increased, respectively. Coxa valga and failure of acetabular development is accompanied by femoral head subluxation. The hip dysplasia results in hip pain and limited range of motion in the early period. The coxa vara deformity is characterized by a femoral neck - shaft angle less than 110°. This can limit the hip motion and cause walking difficulties which disturb the daily activities. Both the hip dysplasia and coxa vara deformity can be corrected by utilizing different osteotomy techniques in the acetabulum and the proximal femur. It should be kept in mind that both pathologies related to hip may end up with early hip arthritis, and total hip arthroplasty may eventually be necessary for these patients despite a properly applied hip reconstruction.