Isolated congenital elbow contracture is a rare upper-extremity disorder and there are few data about management of this condition. Authors report their experience after aggressive management of children with isolated congenital elbow contracture in flexion. Because of total absence of range of motion (ROM) improvement despites physical therapy (ROM 90–120°) and bone deformity, an anterior surgical release of the elbow was performed through an extensive lateral approach, at sixteen months of age. After surgery, this child was treated by three casts at maximal gained extension followed by sequential Turnbuckles splints. After five years of follow-up, the result was excellent with ROM 5–135°, normal function and absence of growth disturbance. The limiting factor of this protocol was excessive traction in elbow extension on the neurovascular structures, especially the radial nerve. This treatment represents an aggressive management with multiple general anaesthesia, but was found to be a valid option.