Objectives The main aims of this study were to classify patients with hypothalamic hamartoma (HH) based on neuroimaging features and describe the clinical manifestations of HH. Materials and methods A retrospective review of 214 consecutive patients with HH treated in Beijing Tiantan Hospital was performed. Results HH were diagnosed and divided into Types I-IV based on MRI. Types I and II were defined as the HH attached to the floor of the third ventricle with narrow (Type I) or broad (Type II) interfaces. Type III (straddling') was defined by the HH extending into the third ventricle and interpeduncular cistern. Type IV was defined as the HH located totally within the third ventricle. The percent distribution of patients was 35.9% Type I, 12.1% Type II, 40.7% Type III, and 11.2% Type IV. The percentage of patients with precocious puberty was highest in Type I (81.8%). The percentage of patients with gelastic seizures was highest in Type IV (91.7%). After surgery, 20% (1/5) of patients with Type II HH, 48.8% (20/42) with Type III, and 91.7% (11/12) with Type IV were free of epileptic seizures. Significant prognostic factors for surgical outcome were HH size, surgical approach, and resection level. Conclusions The clinical manifestations of HH are correlated with the topology of the HH in relation to the hypothalamus. Our results suggest that patients with Type IV HH have the best outcome from surgery and neurosurgeons should be cautious about performing surgery on patients with Type II and Type III HH.