Syndrome of hepatic cirrhosis, dystonia, polycythemia, and hypermanganesemia caused by mutations in SLC30A10 gene, a Manganese transporter in humans  has been recently described. Because of hepatic involvement and extrapyramidal neurological manifestations, many cases are misdiagnosed and treated as Wilson’s disease. We here report a 14-year old girl with manganese transporter defect that resulted from a novel mutation in SLC30A10 gene from Southern India. Our patient, 2^nd child born to a 2^nd degree consanguineously married couple developed sub-acute hepatitis at 3-years of age that spontaneously resolved with supportive care. Three months later she developed insidious onset gradually progressive extrapyramidal features like drooling and feeding difficulties with abnormal posturing of limbs (right more than left). At 12-years, she developed dyspneoa secondary to polycythemia and underwent bloodletting twice. There was no evidence of KF ring in the cornea. Her MRI brain showed T1 hyperintensities (T2 isointense) in the basal ganglia, brainstem and cerebellum which were suggestive of manganism. Her manganese levels were elevated (9.8 microgm/l; range: 0.3-1.8)and genetic analysis showed a novel homozygous truncating mutation in the SLC30A10 gene there by confirming the diagnosis of Managenese transporter defect. She was treated with antidystonic drugs and iron supplementation along with physiotherapy. Because of unavailability of BAL and disodium calcium edetate antichelation therapy could not be offered. There was no further worsening (for last 12-months). To conclude, suspect manganese transporter defect in a child with hepatic involvement, extrapyramidal presentation and polycythemia with signal changes in the T1 weighted images in basal ganglia.