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A 52-year-old woman developed episodic diplopia during a period of six months. Between the episodes, depression of the right eye was mildly impaired and lid lag was evident in the right eye on downward gaze. On resuming the primary position after prolonged leftward gaze, she developed a right esotropia and limitation of abduction, elevation, and depression in the right eye. Lid lag may be a sign suggesting oculomotor neuromyotonia in patients with episodic diplopia. The isolated lid lag without lid retraction suggests primary aberrant oculomotor regeneration as a mechanism of this phenomenon.