The primary objective of the study was to compare the trace element status in children with infantile tremor syndrome (ITS) with healthy controls. Blood counts, serum vitamin B12, plasma homocysteine, plasma ferritin and trace elements - magnesium, zinc and selenium were compared. Twenty-six children (11 with tremors, 14 boys, mean age 11.5 ± 3.5 mo) and 19 controls were enrolled for this study. Low vitamin B12 levels (67% vs. 5%) and elevated plasma homocysteine 96% vs. 26%) was significantly more (P < 0.001) in cases. Eight controls had hypoferritinemia, while none with ITS had it (p < 0.001). None of the children in either group had low serum levels of zinc or magnesium. Low levels of selenium were seen in six cases and five controls. This study did not show any association between the studied nutrients/ trace elements and ITS while, it showed a strong association of ITS with vitamin B12 deficiency supporting that it is a Neurocutaneous Infantile B-12 deficiency (NIB) syndrome.