We present a 9-year-old boy with central precocious puberty and hydrocephalus caused by a human chorionic gonadotropin (beta-hCG) secreting pineal tumor. High levels of beta-hCG in the serum and cerebrospinal fluid were observed in this patient. The patient received radiotherapy and chemotherapy without surgical intervention. Subsequently, significant tumor regression was observed and the serum beta-hCG level normalized. There was no evidence of tumor recurrence at follow-up one year after treatment. The role of tissue biopsy to establish a diagnosis in pineal germ cell tumors remains controversial because it can be a difficult procedure and may be dangerous, causing severe complications. In this patient, the elevated beta-hCG level indicated the presence of a tumor and was considered sufficient evidence to warrant initiating treatment.