Abstract:Introduction: Congenital anomalies affect 1% to 2% of newborns, and approximately 10% of those children have upper-extremity abnormalities. The hemimelias can have three additional descriptions: complete, partial and paraxial.Case Report: We present a rare case of 4 year old female child who presented to our department with the absence of distal humerus, elbow joint, forearm and hand since birth. On examination, the patient was found to have a small nevus about 0.5 cm in diameter over the thoracic spine and bilateral flat feet with metatarsus adductus of left limb. The child also had weakness of the right abdominal musculature. Radiograph of the upper limb was taken which showed absence of the distal humeral epiphysis. Conclusion:Early use of prosthesis in congenital amputee can help in reducing the disability for the child in the future and help the child to have a near normal functioning limb.