Peutz-Jeghers Syndrome (PJS) is a rare genetic disorder characterized by distinctive mucocutaneous pigmentation and gastrointestinal polyps. It is inherited in an autosomal dominant manner, with mutations in the serine/threonine kinase 11 (STK11) gene identified as the primary causative factor. PJS poses a complex challenge due to its varied clinical manifestations and increased cancer predisposition. Early diagnosis and appropriate management are crucial for improving outcomes for affected individuals. Management aims to address symptoms, prevent complications, and reduce the risk of cancer through vigilant surveillance and appropriate interventions. Proper nutrition, regular physical activity, and routine medical check-ups are crucial for managing PJS. Pharmacological and non-pharmacological interventions, as well as surgical intervention, may be necessary depending on the specific complications. This document explores the role of pharmacists in understanding, treating, and supporting individuals with PJS. Pharmacists play a crucial role in providing clear medication instructions, lifestyle recommendations, and educational materials to optimize health outcomes. They offer guidance on sleep hygiene, health promotion, disease prevention, psychosocial support, over-the-counter medication usage, iron supplementation, and drug-interaction checks. By empowering patients to take an active role in their health and facilitating support networks, pharmacists contribute significantly to the holistic management of Peutz-Jeghers Syndrome.