Orbital xanthogranuloma is a rare dermatological condition characterized by its bilateral and rather symmetrical subcutaneous granulomatous infiltration around the eyes. It shares morphological similarities with other xanthogranulomatous diseases such as juvenile or adult type xanthogranuloma, necrobiotic xanthogranuloma and cutaneous infiltration in Erdheim-Chester disease, and should be differentiated from them. Three elderly Chinese patients with this condition were found to have underlying haematological abnormalities: normochromic normocytic anaemia, thrombocytopenia or eosinophilia. Their skin lesions remained localized and associated haematological abnormalities stable for up to 7 years. Haematological abnormalities were also reported in previous cases and this finding is unlikely to be coincidental; however, its clinical significance remains unknown.