Objective: To analyze the clinical characteristics, diagnosis, treatment and prognosis of patients with multisystemic sarcoidosis in order to improve the clinical diagnosis and treatment. Methods: A multisystemic sarcoidosis patient presenting with cranial nerve involvement as the first symptom was admitted to Beijing Hospital, and the clinical manifestations, imaging and pathological features were analyzed.And the related literatures were reviewed and summarized. Results: The sarcoidosis patient presented with weakness in the limbs, bilateral paralysis, visual floaters and subcutaneous masses.Chest CT showed multiple symmetrical lymph nodes in the mediastinum and bilateral hilum, with multiple ground-glass and grid-like densities in both lungs.The subcutaneous mass biopsy showed non-caseous granulomas, which was often considered as sarcoidosis.The condition improved after glucocorticoid treatment, while it relapsed after drug withdrawal, with new-onset of shoulder and knee pain.The endobronchial biopsy showed non-caseous granulomas.And the symptoms of the patient were relieved after the retreatment with glucocorticoid. Conclusions Multisystemic sarcoidosis is a rare clinic entity, and cranial nerve involvement as the first symptom is rarer.The diagnosis and treatment of multisystemic sarcoidosis are often delayed due to atypical symptoms.Although patients with sarcoidosis are sensitive to glucocorticoid therapy, those with extra-pulmonary involvement are more likely to relapse.Therefore, not only standardized treatment but also continuous monitoring of disease changes must be needed in clinic.