History and clinical findings: The case of a 70 year old male is presented. Past medical history revealed the following diagnoses: liver cirrhosis, splenomegaly, thrombocytopenia, macrocytic anaemia, chronic obstructive pulmonary disease, type IIa diabetes mellitus and peripheral vascular disease of the lower limbs with chronic leg ulcers. Within 6 weeks the patient developed an increasing number of pigmented lanugo type hair over both cheeks, zygomatic arches and temples. The diagnosis of circumscribed acquired hypertrichosis lanuginosa (AHL) was established. Bone marrow biopsy revealed presence of acute myeloid leucaemia. Therapy and clinical course: Chemotherapy with Hydroxycarbamide (Syrea((R))) was started without effect on haematologic parameters. The patient was transferred to the oncology unit where he deceased soon afterwards. Conclusion: Acquired hypertrichosis lanuginosa (AHL) is a paraneoplastic syndrome. It is mainly associated with adenocarcinoma of lung and bowel, however other histologic types of tumours and localisations have been described. About 50 cases have been reported in the literature until 1998. To our knowledge this is the first case of AHL described in association with acute myeloid leucaemia.