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Metaphyseal chondrodysplasia is a very rare autosomal dominant disorder affected of enchondral ossification in especially metaphysis (1). Firstly described in 1934 by Murk Jansen as various severity and degrees (2), and metaphyseal chondrodysplasia was classified in 1957 by Weil. Jansen type, which is the one of the most serious and rare. Hyperkalemia and hypophosphatemia were seen half of these patients (3,4). In this article we intended to draw attention to some issues related with anesthesia management that a 9 year-old patient a with Jansen’s metaphyseal dysplasia which to be a disorder very rare and hardest diagnosing.

Anesthesia management of Jansen’s metaphyseal dysplasia