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Objectives: The diagnosis of early onset of myasthenia gravis in the emergency room represents a real challenge, especially when symptoms of presentation are atypical and nonspecific features. Material and methods: We described a case of 33 years old woman who went to the Emergency Department of Pescia (Pistoia, Italy) for a spontaneous intense frontal headache associated with the presence of rhinolalia and unilateral shoulder girdle disorders, initially misdiagnosed. Results: The atypical and initially mild presentation of the disease contributed to the delay in diagnosis; the progression of motor disturbances subsequently led to the definition of the clinical pattern. In particular, the patient showed AChR antibody positivity, associated with a thymoma. Conclusions: Despite being a well-known pathology and widely described in the literature, myasthenia gravis at onset is still a difficult diagnosis in cases of atypical presentation or with infrequent symptoms. [ABSTRACT FROM AUTHOR]