AbstractWe report an atypical granular cell tumour (GCT) presenting in a 6‐year‐old boy. GCTs are of neural origin and most cases arise in patients between the ages of 40 and 60. There are few reported cases in children, in whom malignant presentations are exceptionally rare. This patient presented with a 1 year history of a slowly enlarging nodule on the right anterior abdomen. Examination revealed a firm, nodular dermal skin lesion, which was fully excised. Histology revealed an atypical GCT.