Japan is one of the rare non‐tropical countries with documented cases of Buruli ulcer (BU). Mycobacterium ulcerans subsp. shinshuense has been identified as the causative agent. The first report of BU in Japan dates back to 1982, with sporadic reports thereafter. Recently, the number of cases has been on the increase, and 50 cases (57.7%) are from the past decade alone, out of a total of 87 cases reported to date. Japan's well‐developed healthcare facilities play a crucial role in enabling detailed investigations and providing appropriate treatment for patients, contributing to a favorable prognosis. However, the rarity of the disease results in lack of awareness among healthcare professionals, leading to frequent delays in diagnosis. This article aims to offer an updated overview of BU cases in Japan and to raise awareness of BU among dermatologists and other healthcare professionals in a non‐endemic setting.