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Purpose Common variable immunodeficiency disorders (CVID) are a group of primary immunodeficiencies characterized by impaired immunoglobulin production and dysregulated immune response. Neurological manifestations have been described in few patients, and little is known about its clinic and therapeutic approach. Thus, the aim of this work was to review the literature on it and to help differentiate CVID from its mimics, especially sarcoidosis. Methods We described a case report and included a literature review of inflammatory neurological involvement in CVID. Results A 32-year-old female patient with a medical history of recurrent bacterial infections, temporal focal epilepsy and granulomatous lung disease under study, and cervix squamous cell carcinoma, was initially admitted to the emergency department due to intracranial hypertension. After excluding infectious and neoplastic etiologies, the most likely hypothesis was that granulomatous pulmonary, cerebral, and leptomeningeal inflammatory involvement were associated with sarcoidosis. Two years later, a diagnosis of CVID was made, and the patient was secondarily diagnosed with Granulomatous and Lymphocytic Interstitial Lung Disease (GLILD) and related inflammatory brain disease – both complications of CVID. After starting targeted treatment with immunoglobulin replacement and pulse glucocorticoids followed by a chronic taper, the patient became stable. However, three consecutive failures in immunoglobulin intake led to disease recurrence with relapse of neurological manifestations. Conclusion This case illustrates the complex multiple organ manifestations of CVID. When granulomatous conditions arise in these patients, GLILD with multisystem involvement should be taken in consideration. Early treatment with combined steroids and immunotherapy seems to be effective in controlling CVID’s neurological manifestations.