Gaucher disease represents the largest lysosomal storage disease group worldwide. Possible complications include the development of Gaucheromas, pseudotumors resulting from an accumulation of Gaucher cells. Gaucheromas can affect the liver, spleen, bones, and lymph nodes. Descriptions of the appearance of lymph node gaucheromas exist for computed tomography (CT) and magnetic resonance imaging (MRI) but not, to our knowledge, of their ultrasound characteristics. We present the case of a four-year-old boy with Gaucher disease with lymph node Gaucheromas, discovered during a routine follow-up, and present their ultrasound characteristics. We describe characteristic ultrasound findings of non-B-cell lymphomas and Gaucheroma lymph nodes in comparison.Teaching point:Lymph node Gaucheromas have a characteristic ultrasound appearance and should be searched for in Gaucher’s patients.