Differences in the epidemiology, diagnosis and outcomes according to gender in patients diagnosed with non-muscle invasive bladder cancer (NMIBC) has been widely reported. In this article we present gender-specific differences in NMIBC in terms of epidemiology, risk factors, first clinical presentation, management and clinical outcomes based on systematically review evidence of existing literature. A literature search of English-language publications that included an analysis of the association of gender differences in patients with NMIBC was performed using PubMed. Sixty-four studies were selected for analysis with consensus of all authors. The incidence and mortality for urothelial bladder cancer (UBC) are higher in men, whereas cancer specific mortality to incidence ratio is significantly lower for men than for women. This phenomenon could be partially explained by differences in exposure to bladder cancer carcinogens. However female gender is associated with higher stage at presentation. Thirteen studies with a total of 11,069 patients diagnosed with NMIBC were included for analysis according to outcomes. In studies that found statistically significant differences in outcomes between sexes, female gender was reported as risk factor for disease recurrence, progression or cancer specific mortality. None of included studies found worse outcomes in men when compared to women with NMIBC. Results of our review suggest that female gender in patients diagnosed with NMIBC is associated—though inconsistently—with higher stage at presentation and poorer outcomes. Numerous factors may influence gender gap in incidence rate, clinical management and reported outcomes. Consensus on comparable data collection in routine practice and prospective trials including clinical outcomes are required to identify gender-specific differences in patients diagnosed with NMIBC.