A child with intermittent upward deviation of the eyes starting at 9 months of age, compensating bending forward of the head, nystagmus on attempted downward gaze and a mild gait ataxia is described. The symptoms gradually disappeared between 3 and 4 years of age. Four cases with identical clinical findings have been initially described by Ouvrier in Australia (5) as "benign paroxysmal tonic upgaze of childhood". It is apparently a new syndrome.