Objective: Gestational trophoblastic disease (GTD) is a peculiar disease in females of reproductive age group because of their natural history, management, high potential for bleeding and excellent response to chemotherapy. Although the incidence of brain metastases is only 10%, propensity for uncontrollable hemorrhage in brain parenchyma makes this situation, a medical emergency. We discuss case series of 7 patients with high risk GTD with brain metastases and their management. Study Design: A retrospective analysis of 15 years from January 1996 to December 2010 was done to study all cases of GTD treated in this institution. Patients who presented with brain metastases on initial diagnosis (early group) and who had metachronous disease (late group) during the course of treatment or follow‑up were summarized in this study. Materials and Methods: Of 1208 cases of hydatidiform mole treated in this institution, 325 cases had low risk (WHO score 6 or less), and 38 patients had metastatic disease (score 7 and more). Seven cases were diagnosed to have a high risk GTD with brain metastases, 5 (71.43%) presented initially and 2 (28.57%) developed late brain metastases. Results: In our analysis, patients who had brain metastases on presentation had better survival (median = 52 months, range = 18‑61) compared with patients who developed brain disease later in the course (median = 5.5 months, range = 3‑8). Conclusion: Gestational trophoblastic disease with early brain metastases presentation showed better response and survival compared with late presentation group.