BACKGROUND Bilateral congenital absence of femur is a rare congenital anomaly. METHODS We report a 2-year-old Indonesian female child, the third of the birth of diabetic mother. There is no congenital abnormality among her siblings. She has a normal mentality and normal upper limbs. She was brought to us with a complaint of the short lower limb. Examination revealed short lower limb with an absence of thigh, a full range of motion of the hip and ankle and left club foot. A detailed history was obtained and she was radiologically examined. The X-ray of the pelvis and lower limb shows shallow of the acetabulum, absence of bilateral femur, and uncommon form of tibia fibula. She was diagnosed with bilateral congenital absence of femur. CONCLUSIONS We report this case because it does not meet the existing classification criteria for congenital femur deficiency. Proper evaluation and management of this case can help the patient and the parents to accept her condition and be able to live a good social and economically productive life.