Spitz naevus is an uncommon, benign melanocytic neoplasm that shares many clinical and histological features with melanoma. While Spitz naevi typically occur in children and melanomas typically occur in adults, either tumour can occur in a patient of any age. In cases displaying all or most of the classical histological features, particularly when occurring in a young patient, it is possible to make a confident diagnosis of Spitz naevus. However, for those lesions with atypical features it may be difficult to predict the biological behaviour with certainty from the histopathological appearance. Hence, such tumours have been referred to by a variety of names including atypical Spitz naevus, atypical Spitz tumour and spitzoid tumours of uncertain malignant potential. However, even acknowledged experts in dermatopathology have a low concordance in distinguishing Spitz naevus with atypical features from melanoma. In this article, we highlight the histopathological features of Spitz naevi and those that may be useful in distinguishing Spitz naevi from melanomas. A suggested practical guide to clinical management of such lesions is provided.