Isolated hypoglossal nerve palsy is rare, but occasionally it appears as the initial or solitary sign of an intracranial or extracranial space-occupying lesion or a vascular abnormality of the internal carotid artery. We present a 14-year-old girl who, following an upper respiratory tract infection, presented with isolated right hypoglossal nerve palsy. Anti-streptolysin O titre was increased to >1280 suggesting a preceding streptococcal infection. Magnetic resonance imaging of the brain did not show any intracranial or extracranial abnormality. She had a partial improvement at 3 months. This case emphasizes the value of recognizing the existence of benign self-limiting, post-infectious causes of isolated hypoglossal nerve palsies in children.