Sentinel lymph node biopsy has been proposed as a diagnostic method for estimation of the malignant potential of atypical Spitz tumours. However, although cell deposits are commonly detected in the sentinel lymph nodes of patients with atypical Spitz tumours, their prognosis is substantially better than that of patients with melanoma and positive sentinel lymph node biopsies. We did a systematic review of published reports to assess the role of sentinel lymph node biopsy as a prognostic method in the management of atypical Spitz tumours. The results of our analysis did not show any prognostic benefit of sentinel lymph node biopsy; having a positive sentinel lymph node does not seem to predict a poorer outcome for patients with atypical Spitz tumours. These findings indicate that, especially in the paediatric population, it might be prudent initially to use complete excision with clear margins and careful clinical follow-up in patients with atypical Spitz tumours.