Amyloidomas of the nervous system are rare in clinical practice and rarely reported in the literature. Primary intrasellar amyloidomas are even rarer. We encountered a 39-year-old male patient, whose chief complaint was headache and who had been hospitalized for 10 days. The patient did not have any past medical history. Preoperative computed tomography and magnetic resonance were nevertheless performed to exclude the possibility of pituitary apoplexy or Rathke cleft cyst. Due to the progressive worsening of his headache, a neuroendoscopic transsphenoidal tumor resection was carried out. During surgery it was found that the tumor exhibited a yellow crystalline substance with an inadequate blood supply and no obvious encapsulation. The tumor was completely resected under endoscopy, the amyloidoma was confirmed by postoperative pathological results, and no pituitary adenoma was found. The patient's headache resolved after surgery, and after a 1-year follow-up, the tumor had not recurred.