Benign paroxysmal torticollis of infancy is a benign, rare, probably under-recognised disorder, characterized by recurrent episodes of head tilting. The diagnosis is primarily one of pattern recognition and exclusion of alternatives conditions; other symptoms, such as vomiting, pallor and eyes' rotation, may be associated with or rapidly follow the attack, leading to misdiagnosis of this disease. The exact pathogenesis of benign paroxysmal torticollis is not clear, but a close relationship with childhood periodic syndromes is supposed. Due to the difficulty in defining the disease, this event has implications with respect to the training and education of practice providers and emergency physicians. We describe the case of a 7-month-old infant with benign paroxysmal torticollis recently observed, discuss the clinical presentation and review the literature.