Mannosidosis is an extremely rare genetic disease occurring due to deficiency of the lysosomal enzyme, alpha-mannosidase. Patients with this disorder often suffer from musculoskeletal abnormalities and muscular weakness leading to joint destruction and severe morbidity along with other major systems involvement. We present here such a case of a 27-year-old male that highlights the challenges in management of hip joint destruction secondary to Mannosidosis.
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