A 26-year-old female with hereditary neutropenia had Pustulosis Palmaris et Plantaris. Drug administration did not improve her symptoms. Following administration of granulocyte colony stimulating factor (G-CSF; filgrastim), her neutrophil count increased from 300 to 58,000/microliters, and her dermatosis improved. Pustulosis Palmaris et Plantaris has been described as a representative second lesion of focal infection. Neutropenia may be one cause of Pustulosis Palmaris et Plantaris due to refractory focal infection. In the present case, increase in neutrophils with G-CSF may have improved focal infection, resulting in improvement in refractory Pustulosis Palmaris et Plantaris.