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Nephrotic syndrome (NS) is a common glomerular disease characterised by massive proteinuria, hypoalbuminaemia, oedema and hyperlipidaemia. Cerebral venous sinus thrombosis (CVST) is a rare complication in children with NS. Here, we report a case of relapsing NS on steroid therapy, in a male in early childhood who presented with symptoms of headache, vomiting and double vision. On prism cover test there was 25 PD esotropia with abduction restriction in the left eye. Fundus examination showed bilateral papilloedema. He was diagnosed as sixth cranial nerve palsy of left eye. Neuroimaging reported dense CVST. He was managed with subcutaneous low molecular weight heparin and steroids. After 2 months of treatment, there was a complete resolution of esotropia and optic disc oedema. This case highlights the importance of early diagnosis of acute onset esotropia and sagittal sinus thrombosis in a case of NS.