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Neglected tropical zoonotic diseases (NTZDs) continue to affect the health and livelihoods of humans particularly the poor and marginalized populations in developing countries. Mapping the distribution and burden of these diseases will support making an informed decision. A retrospective study was conducted to map the spatial distribution and analyse trend of NTZDs in Tigray region, Northern Ethiopia. A health management information system (HMIS) data between 2012/13 and 2016/17 was obtained from Tigray National Regional State Health Bureau. The Quantum‐GIS software was used to map the spatial distribution and burden of selected NTZDs at zonal level. Only four of the NTZDs namely rabies, tuberculosis (TB), schistosomiasis and visceral leishmaniasis (VL) from the records of HMIS data were considered. A high incidence rate (IR) at least for one of the NTZDs was reported in males above 15 years old. In the 5‐year period, of the 60,099 reported NTZD cases, 30.3% (18,220), 26.6% (16,005), 23.3% (14,001), 14.4% (8,661), and 5.3% (3,212) were, respectively, schistosomiasis, extra‐pulmonary TB, rabies, pulmonary TB, and visceral leishmaniasis. The year‐wise analysis showed an irregular trend for the NTZDs where both the decreasing and increasing trends didn't show statistically significant variation. However, the overall regional number of TB cases showed a decreasing trend, where the decrease for extra‐pulmonary TB (1.8 cases per 100,000 population) was higher than pulmonary TB (0.5 cases per 100,000 population). Similarly, the annual number of rabies and VL cases showed a decreasing trend. On the other hand, the number of schistosomiasis cases showed an increasing trend (8.2 cases per 100,000 population). The annual average number of TB (171 cases per 100,000 population), schistosomiasis (354 cases per 100,000 population), and VL (63 cases per 100,000 population) cases were much higher in Western zone compared to the rest of the zones. The incidence rate of rabies was higher in Mekelle and Southeastern (100 cases per 100,000 population) and Northwestern (97 cases per 100,000 population) zones. Intervention strategies applied in the region should take into account the zonal distribution and burden of NTZDs. [ABSTRACT FROM AUTHOR]