Purpose: To report the possible relationship between otologic pneumocephalus and the Valsalva maneuver in a patient at our institution with a brief literature review. CASE REPORT: A 38-year-old HIV-positive man undergoing antiretroviral therapy presented with a 2-year progressive hearing loss in his left ear. He had a feeling that his ears were plugged and used to perform multiple Valsalva maneuvers daily. He did not have fever, pain, dizziness, or tinnitus. A computed tomography (CT) scan revealed left mastoid hyperpneumatization with foci of bone discontinuity in the mastoid inner cortex associated with homolateral adjacent epidural pneumocephalus. Despite medical advice, the patient continued to perform various Valsalva maneuvers daily, and a follow-up CT scan 10 months later showed increased pneumocephalus. CONCLUSION: Some reports demonstrated that the Valsalva maneuver may cause mastoid hyperpneumatization and pneumocephalus. Our report reinforces this association and is very important for the therapeutic approach in such patients.