Objective To summarize the clinical characteristics of patients with basal ganglia germinoma (BGC). Method The general information, clinical manifestations, laboratory examination and imaging results of seven patients with BGC were retrospectively analyzed. Results A total of seven patients diagnosed with BGC were retrospectively analyzed, including six males and one female, with a median age of ten years and eight months. The median time from onset to diagnosis was three months. The most common clinical manifestations were lateral limb weakness. Alpha-fetoprotein and beta-human chorionic gonadotrophin were normal, and specific oligoclonal bands in cerebrospinal fluid was positive in some patients. The common imaging findings were unilateral basal ganglia atrophy accompanied by cerebrum atrophy, and bilateral basal ganglia can also be involved. All the seven patients were confirmed by stereotactic biopsy histopathology. Conclusions BGC is characterized by lateral limb weakness and early imaging findings were atypical. The imaging features are the basal ganglia and cerebral peduncle.