Porcelain gallbladder (PGB) has historically been considered a premalignant condition, although recent evidence questions the strength of its association with gallbladder carcinoma. Objective: To describe the occurrence and characteristics of malignancy in patients with porcelain gallbladder, as well as clinical, imaging, and surgical characteristics reported in clinal cases. Methods: A systematic review of case reports and case series (≤5 patients) was conducted following PRISMA 2020 guidelines. PubMed/MEDLINE, ScienceDirect, and Redalyc from database inception through March 2026. Individual patient data were extracted including demographic characteristics, imaging findings, calcification pattern, surgical management, histopathology, and outcomes. Descriptive statistics were performed. Results: Fifty-four individual cases of porcelain gallbladder were identified. Patients were predominantly female (70.4%), with ages ranging from 6 to 98 years. Complete calcification was reported in 85.2% of cases. Histopathological confirmation was available in 45 cases (83.3%). Thirteen patients had gallbladder carcinoma associated with porcelain gallbladder, corresponding to 28.9% of those with pathological examinations. Incomplete calcification showed higher frequency of malignancy compared with complete calcification. Mortality was reported in three patients (5.6%), all with malignant disease. Conclusions: The proportion of malignancy observed in published case reports reflects a selected dataset and should not be interpreted as an estimate of population risk. These findings provide descriptive insights into clinical presentations and management patterns, supporting hypothesis generation rather than risk estimation.