Upper limb dysfunction, particularly in relation to skeletal dysplasia and median nerve dysfunction, is well recognized in the mucopolysaccharidoses and mucolipidoses. This is of increasing importance because bone marrow transplantation has dramatically improved survival without changing the musculoskeletal manifestations. Triggering at the A1 pulley and more distally has been described in these patients. We report two such cases and four other patients who were noted to have triggering at the carpal tunnel in association with carpal tunnel syndrome. Five of the six patients consented to surgical intervention with favorable outcomes.