Here we describe an attenuated juvenile onset form of mucopolysaccharidosis plus that is due to inheritance of a previously unreported homozygous variant p.Arg200Pro in VPS33A protein, a core subunit of the HOPS and CORVET tethering complexes identified in an adolescent patient with long‐standing intellectual disability, skeletal deformities, and evidence of mild cardiac disease (Dursun et al., 2017; Kondo et al., 2017; Pavlova et al., 2019). This evidence concerns the gene VPS33A and mucopolysaccharidosis.