Pseudotumor cerebri is a clinical syndrome characterized by raised intracranial pressure with normal ventricular size, anatomy and position. Headache, vomiting and diplopia are the most common symptoms. Signs include those of raised intracranial pressure including papilledema and absence of focal neurological signs. A secondary cause is identifiable in 50% of children; the most common predisposing conditions are otitis media, viral infection and medications. Management is mainly directed towards identifying and treating the cause and measures to reduce the raised intracranial pressure. Though it is mostly a self limited condition, optic atrophy and blindness can occur. Oculomotor nerve palsy is very rarely associated with pseudotumor cerebri. We report a unique case of pseudotumor cerebri who had left Oculomotor palsy with sparing of the pupillary fibres, which resolved following treatment with oral acetazolamide.