We present the case of a young boy with Dent’s disease, identified as having a mutation in the kidney-specific chloride-proton antiporter CLCN5 during investigation for nephrotic range proteinuria. He went on to develop a growth hormone deficiency requiring treatment with recombinant growth hormone followed by an acute presentation with hepato-renal failure and thrombotic occlusion of both middle and right hepatic veins consistent with a diagnosis of Budd Chiari syndrome, which required a prolonged period of intensive care. We have identified 3 reports in the literature in which growth hormone therapy has been used to treat short stature associated with Dent’s disease. This report confirms that growth hormone deficiency is a recognised finding in this rare disease. There are no previous reports on thrombotic complications associated with either Dent’s disease or the use of recombinant growth hormone per se. The cause of the Budd-Chiari syndrome in this case has yet to be fully elucidated, but potentially widens the Dent’s phenotype and gives support to the previous observation that this disease is multifaceted with a possible role for as yet unidentified environmental and/or genetic modifying factors.