Epiphyseal separation of the distal humeral epiphysis is an extremely rare injury. Both clinical and radiological examinations are unclear and delayed diagnosis is reported. The absence of the ossification center of the capitellum complicates the radiological diagnosis and often it is reported as elbow dislocation. We are reporting a case of a distal humeral epiphysis separation, diagnosed in the maternity hospital. The neonate had severe discomfort and reduced mobility of the affected arm. We confirmed the diagnosis with the conventional radiological examination, with the medial displacement of the olecranon and the articulation of the radius with the medial humeral condyle. We performed immediate reduction of the lesion and evaluated the restoration of the normal elbow anatomy with the correct articulation of the radius with the lateral humeral condyle. The neonate was followed for a year and there is normal development of the affected elbow. Knowledge of this rare neonatal fracture is essential to diagnose properly the lesion. Plain radiographic evaluation enabled us to diagnose the epiphysiolesthesis. We have treated our neonate with no invasive procedures and the final result was the normal development of the elbow joint.