The congenital femoral deficiency is considered an uncommon disease in dogs and cats that may be uni- or bilateral. Therefore, the aim of this report is to describe the clinical signs and imaging findings in a dog with proximal femoral focal deficiency. A 10-month-old, entire male, Labrador retriever dog was presented due to lameness in the right hind limb. There was no history of trauma. On gait evaluation, the dog showed a severe weight-bearing lameness in this limb. Physical examination showed mild crepitation and discomfort only during abduction in the right hip joint. The right hind limb was shorter than the left. Plain radiography and computerized tomography imaging of the hind limbs showed a small femoral head grossly deformed and dislocated craniodorsally, aplasia of the femoral neck, and right femur 5 cm shorter than left femur (27% of length discrepancy). Other radiographic abnormalities were not observed in the hind limbs. The owner refused to have any surgical procedure performed. In human patients, the disease is associated with thalidomide, but other causes have also been suggested. The cause of the disease is unknown in animals, but apparently is not hereditary. In the present case, the owner had no information about the other littermates.