The association of abnormal granulocyte nuclear formation and tuberculosis was first reported by Pelger in 1928. Huet, however, concluded this defect was hereditary, and the association of Pelger-Huet anomaly (PHA) of granulocytes and tuberculosis was obscured for many years. We report the second patient in the English literature with severe tuberculous infection and PHA, further substantiating the diagnostic and prognostic importance of PHA and tuberculosis.