Radial Ray Defect (RRD) is a rare disorder, and the etiology of this disorder is still under discussion. RRD is associated with many medical conditions for which prenatal counselling is of paramount importance. Any association with the family history is still unknown. The patient is a 16-year-old female who came to the orthopaedic clinic complaining of tingling and weakness in the right forearm. On examination, there was a gross deformity in the right forearm with radial deviation of the hand. An X-ray revealed radial ray defect type-2. She previously had a history of perinatal infection during early childhood. Surgery was successfully achieved, and positive results were accomplished. Radial Ray Defect can be focal or associated with other clinical manifestations. The timing of antenatal ultrasound for detecting this musculoskeletal anomaly is crucial. When RRD is associated with other syndromes, counselling to the parents about the quality of life and morbidity comes into play. Treatment is primarily surgical.