Imerslund-Najman-Grasbeck disease is a rare inherited megaloblastic an
aemia secondary to a selective malabsorption of vitamine B12 by ileal
enterocytes. The authors report on a 4 year-old tunisian girl who pres
ented as visceral infantile leishmaniasis because of huge splenomegaly
and major anaemia The diagnosis of Imerslund disease was performed on
the basis of the association of typical megaloblastic cells in the ma
rrow, permanent proteinuria and favourable outcome under parenteral B1
2 administration. In addition, ther were no folate deficiency, no anti
-intrinsic factor antibodies and no intrinsic factor deficiency. The o
utcome of the disease is always favourable if parenteral administratio
n of vitamine B12 maintained.