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Background: Inflammatory pseudotumor is a non-neoplastic process of unknown etiology characterized by proliferation of connective tissue with an inflammatory infiltrate. IPT most frequently arises in the orbit, but can also be found in the larynx, the paranasal sinus and rarely in the skull base. Case report: We present the case of a 20-year-old patient with a 4-month history of headache and insomnia. Neurological examination showed limited head mobility and hypoglossal nerve dysfunction. The patient was afebrile and no abnormalities in blood tests were found. CT revealed the presence of a tumor mass destructing the right occipital condyle. MRI was performed and the mass was surgically removed. The histological diagnosis was non-specific chronic inflammatory granulation tissue. Conclusions: Inflammatory pseudotumors can often mimic malignant neoplasms, especially in cases where bone destruction is observed. IPT of the occipital condyle is a rare but aggressive lesion that should be treated by surgical excision.