Lyme disease is endemic in northern Europe and the USA and affects approximately 500,000 people per year. The etiological agent of this zoonosis is a gram-negative spirochete of which approximately 20 different genotypes have been identified to date. Different Borrelia genotypes present a distinct organotropism responsible for the multiple clinical manifestations that are also linked to the different temporal stages of the infection. There is no stereotype that can summarize the signs and symptoms of this infection as it crosses a clinical spectrum that varies from asymptomatic infection to disabling or fatal disease. The paper presents the case of a boy diagnosed and treated for neuroborreliosis. This case is emblematic to highlight and share the heterogeneity of clinical presentations in Lyme disease. A 13-year-old patient, with no history of significant disease, was admitted to the paediatric unit for the sudden onset of right mono-ocular convergent strabismus. Sharing this case aims to highlight the diagnostic-therapeutic paradigm that the presentation of Lyme disease can entail. The issue for scientific discussion is the need for a diagnostic consensus for Lyme disease in the paediatric population.