That high-density lipoprotein cholesterol (HDL-C) is cardio-protective is well known, earning the designation “good cholesterol”. This has led to HDL-C being recognized as a major cardiovascular disease risk factor. Severe HDL-C deficiency is uncommon, and is believed to be primarily due to genetic mutations of lipoprotein metabolism. Rarer still, are acquired forms of severe HDL-C deficiency. We therefore report our experience with such a patient.