MA is associated with neurological features like developmental delay, cerebellar atrophy, ataxia, psychomotor retardation and dysmorphic features, apart from autoinflammatory symptoms The clinical presentation largely depends on the residual activity of mevalonate kinase ranging from virtually undetectable in MA to a range of detectable, but clearly deficient in HIDS and possibly resulting in premature death [5]. The gene discussed is MVK; the disease is microtia.