The following investigations led to the diagnosis: low T cell count with defective response to mitogens suggestive of immunodeficiency, low haptoglobin with positive Coomb’s test suggestive of haemolytic anaemia and increased urinary orotic acid, a known intermediate metabolite of the pyrimidine biosynthetic pathway, suggestive of an alteration of purine salvage pathway. Here, HP is linked to non-autoimmune hemolytic anemia.