Sellar cysticercosis is a rare form of neurocysticercosis. A septum pellucidum cysts is a rare, often asymptomatic cystic structure between the lateral ventricles. We report here a male patient with sellar cysticercosis and septum pellucidum cysts who was successfully treated by neuroendoscopic resection. The patient was a 28-year-old male who presented with intermittent headaches for 5 years. A magnetic resonance imaging (MRI) of the brain revealed a well-circumscribed 13 mm cystic space-occupying lesion in the sellar region and the cavum septum pellucidum. The cyst in the saddle area was completely resected via endoscopic endonasal-transsphenoidal approach. Postoperative histological examination verified cysticerci in the cyst wall. To our knowledge, this is the first case of sellar cysticercosis and a septum pellucidum cyst successfully treated through neuroendoscopic resection.