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Inflammatory pseudotumor (IPT) is a non-neoplastic condition of unknown etiology. IPT with lower cranial nerve (CN IX, X, XI, XII) neuropathies is extremely rare. In this study, we systematically reviewed all previously reported cases regarding the management of IPT with lower cranial nerve neuropathies. We searched the PubMed/MEDLINE database for reports related to IPT with lower cranial nerve neuropathies. A total of six papers with 10 cases met the inclusion criteria (mean age 51.6 years, 70% male). The mean follow-up period was 15.4 months (range: 1–60 months). The most frequent treatment was corticosteroids alone (60%), followed by surgery alone (20%), and multimodal treatment (20%). Corticosteroid therapy was associated with an excellent (100%) response rate at 6 months of follow-up, and half of the patients were in complete remission after 9 months. Both cases who received surgery had persistent neurological deficits. Immediate complete remission of neurological symptoms and resolution on imaging after decompression surgery via an endoscopic endonasal approach (EEA) with corticosteroids was demonstrated in our representative case. This review suggests that EEA is a preferred method for diagnosis and treatment, a promising approach associated with favorable outcomes, and a viable first-line treatment for selected cases, followed by multimodal therapy.