Objective To investigate the clinical and imaging features of hypertrophic olivary degeneration (HOD) secondary to brainstem hemorrhage. Methods The clinical data of one patient with HOD secondary to brainstem hemorrhage was retrospectively analyzed. Results The patient was hospitalized with paroxysmal and body involuntary jitter and other extrapyramidal symptoms. After admission, MRI scan showed bilateral inferior olive nucleus of medulla oblongata were localized hypertrophy. Conclusion The main clinical manifestation of HOD secondary to brainstem hemorrhage is extrapyramidal symptom. The imaging features are abnormal signals and localized hypertrophy at inferior olive nucleus.