Intestinal pseudo–obstructive symptoms, bilateral ptosis and ophthalmoplegia with leukoencephalopathy presenting in childhood are indicative of a multisystem disorder. A condition that can present with this symptom complex is MNGIE syndrome or mitochondrial neurogastrointestinal encephalomyopathy, a rare autosomal recessive disorder of mitochondrial function. Leukoencephalopathy is a hallmark of MNGIE syndrome and was present in all reported cases along with gastrointestinal dysmotility due to progressive degeneration of the muscles of the gastrointestinal tract, weakness of extra–ocular muscles causing ptosis and ophthalmoparesis, peripheral neuropathy and cachexia. Frequent misdiagnoses have been reported and in those patients presenting with ptosis and ophthalmoplegia, it has reportedly been diagnosed and treated initially as myasthenia gravis. Here we present the case of a 5 year old boy presenting with intestinal pseudo–obstruction, bilateral ptosis, myopathic facies, easy fatigability and leukoencephalopathic changes in brain MRI.