The diagnosis and the treatment of dominant strictures (DS) in patients with primary sclerosing cholangitis (PSC) is challenging and the scientific literature on the subject is quite limited. Only level II and level III evidence is available to guide physicians managing patients with DS and PSC. For the diagnosis, intraductal endoscopic ultrasound is the most sensitive (64%) and specific (95%) test. However, the majority of cases require a combination of several different diagnostic tests, as there is no single investigation that can rule out malignancy in this group of patients. For the treatment, serial endoscopic or percutaneous dilatations provide 1- and 3-year biliary duct patency in 80 and 60% of patients, respectively. Dilatation and stenting are the most common interventions, although the optimal duration of treatment has still not been clearly defined. Bile duct resection and/or bilioenteric bypass are currently indicated only for patients with preserved liver function. For all other patients, benign DS can be treated with endoscopic dilatation with short-term stenting. This approach is effective and safe and does not increase the risk of malignant transformation or complications for liver transplant candidates. During the last decade, the use of self-expandable metallic stents for benign diseases has become an innovative option. The aim of this article is to review the diagnostic and therapeutic strategies for patients affected by PSC and DS with specific emphasis on the outcomes of patients treated with temporary stents.