β-human chorionic gonadotropin (β-HCG)-secreting germ cell tumors (GCTs) are rarely identified as the cause of sexual precocity in children. A 9-year-old Taiwanese boy presented with isosexsual precocious puberty associated with an elevated serum β-HCG level. He had no headache, blurred vision, nor any neurologic symptoms and signs. Magnetic resonance imaging of the brain revealed a lesion located in the left basal ganglia. High β-HCG and α-fetoprotein levels were found in both blood and cerebrospinal fluid. Elevation of tumor markers and diagnostic imaging led to the diagnosis of a central nervous system GCT. The patient underwent multiagent chemotherapy plus whole-brain irradiation, and the tumor marker levels returned to normal. In children with advanced puberty in combination with an elevated serum level of β-HCG, malignant secreting GCT should be highly suspected.