A patient with familial cyclic neutropenia (CN) is presented. In a son of a mother with known CN, periodic neutropenia was demonstrated a few months after birth. At 18 years of age, the boy had recurrent episodes of cervical lymphadenopathy, complicated by an abscess. Periodic treatment with granulocyte-colony stimulating factor (G-CSF) was initiated, and since then, he hardly had any symptoms and clinically showed substantial improvement. Based on in-vitro colony assays of patients with CN, it is nowadays suggested that a disturbance in growth factor receptor binding or post-receptor signal transduction is the cause of this rare benign haematological disorder.