This review will address pathological narcissism and narcissistic personality disorder (NPD)-the clinical presentation, the challenges involved in diagnosing NPD, and significant areas of co-occurring psychopathology (i.e., affective disorder, substance usage, and suicide). Major depressive disorder is the most common comorbid disorder in patients with pathological narcissism or NPD. Need for self-enhancement and chronic disillusionment with self make these individuals particularly susceptible to substance use. Suicidal preoccupation in these patients is characterized by the absence of depression, lack of communication, self-esteem dysregulation, and life events that decrease self-esteem. The diagnostic focus on patients' external characteristics and interpersonal behavior tends to dismiss the importance of their internal distress and painful experiences of self-esteem fluctuations, self-criticism, and emotional dysregulation. A collaborative and exploratory diagnostic approach to pathological narcissism and NPD is outlined that aims at engaging the patients and promoting their curiosity, narration, and self-reflection. Alliance building with a narcissistic patient is a slow and gradual process and mistakes are common. A central task is to balance these patients' avoidance and sudden urges to reject the therapist and drop out of treatment with the goal of encouraging and enabling them to face and reflect upon their experiences and behavior. Implications for treatment and possible areas or indications of change include: interpersonal and vocational functioning; sense of agency and self-direction; emotion regulation and ability to understand, tolerate, and modulate feelings; reflective ability; and ability to mourn the loss of wished for or unreachable internal self-states, relationships, and external ideals.