The peripheral smear of a 35-year-old man showed 47% bilobed neutrophils (panel A) and 45% band forms (panel B). A similar smear, had been obtained in his son. A diagnosis of congenital Pelger-Huet was affirmed. Pelger-Huet anomaly is a rare autosomal dominant disorder characterized by pince-nez appearance of the nuclei of the neutrophils. This is caused by failure of nuclear segmentation due to genetic defect in the LBR (laminin B-receptor) on the chromosome 1q41-43. Heterozygotes have dumbbell-shaped or hyposegmented neutrophils with coarse chromatin and normal granules. Homozygotes have neutrophils with a single, round, eccentric nucleus with clumped chromatin and no nuclear segmentation. Pelger-Huet anomaly is a benign condition. An acquired abnormality is referred to as pseudo Pelger-Huet anomaly. This may occur in myelodysplastic disorders, myeloproliferative diseases, and acute and chronic leukemias, reactions to drugs, and so on.