Narcissism is a complex dimension related to the self and characterized by grandiosity and/or vulnerability. Grandiosity is related to explicit dominance, self-importance and entitlement while vulnerability is characterized by the feelings of incompetence, inadequacy and negative affect. According to psychodynamic scholars shame can be considered as the core narcissistic affect, however different dynamics might characterize the experience of shame in grandiose and vulnerable narcissism. Although narcissism and shame have received a certain attention in the literature, a comprehensive review of empirical research examining the relationship between these constructs is still missing. Therefore, our systematic review comprised 25 studies on narcissism in its different manifestations and its relation with shame, as different from guilt. We subdivided our articles in three main clusters: 1) correlational studies of narcissistic personality features, 2) studies on narcissistic personality disorder (NPD) and 3) experimental task studies. Overall our findings show that: i) shame is mainly related to vulnerable narcissism while narcissistic grandiosity seems to be a defensive structure towards threatening affect for the self; ii) guilt doesn’t seem to be consistently related to narcissism across studies; iii) NPD studies confirmed the findings of grandiosity as a defensive structure to preserve the self of the individual, while, on the other hand, when NPD is more vulnerable (e.g., depressive, suicidal, etc.), it seems to be more prone to experience shame; iv) experimental studies investigating narcissism in different scenarios, like performance judgements, dishonesty and spirituality, again confirmed the different dynamics between grandiosity and vulnerability related to shame.