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The authors report on a patient with hereditary osteo-onychodysplasia (HOOD). Osteoporosis was demonstrated upon histomorphologic bone examination. HOOD is a rare inherited disorder. Typical features include dysplasia of the nails, patellae, elbows and ilium. Nephropathy occurs in 48% of patients. Numerous renal histologic studies have led to the concept that the nail-patella syndrome is an inherited metabolic disorder affecting the connective tissue. Our findings from histomorphologic bone assessment bring further evidence in support of this hypothesis.