Granular cell tumours are rare soft tissue neoplasms, which occur at a wide variety of sites and commonly involve the skin. Distinction between benign and malignant granular cell tumours is important because benign tumours can be fully cured by complete excision, whereas malignant tumours commonly recur and cause fatal metastatic disease. Communication between the dermatologist and pathologist is also important, as pathology may provide false reassurance by evaluating a benignappearing part of a clinically malignant tumour. The following review summarizes the current literature on the epidemiology, clinical presentation, pathology, radiology, treatment and prognosis of cutaneous granular cell tumours, with a focus on improving diagnosis and management for dermatologists and dermatopathologists.