Exposure to nickel aerosol in the nickel production is associated with greater occupational risk, yet little is known how many workers will develop an occupational disease and claim compensation. The aim of this analysis was to prospectively observe a cohort of nickel electrolysis workers and quantitatively assess confirmed occupational disease claims. We observed a cohort of nickel electrolysis workers (N = 1397, median age 39, 68% males) from 2008 till 2020 in one of the largest nickel producers in the Russian High North. Cumulative incidence of confirmed occupational disease claims in seven occupational groups, including electrolysis operators, hydrometallurgists, crane operators, final product cleaners, metalworkers, electricians and ‘other’ was analyzed and supplemented with Cox proportional hazards regression, yielding hazard ratios (HR) with their 95% confidence intervals (CI) of occupational disease claims for each group. N patients with occupational disease claims varied from 1 in 2016 to 22 in 2009, and in total 87 patients developed one or more occupational diseases (cumulative incidence 6.2%, p < 0.001 between seven groups). Accounting for 35,527 person-years of observation in total, cleaners exhibited the greatest risk (HR 2.58 (95% CI 1.43–4.64)), also adjusted for smoking, number of non-occupational diseases and group 2 (hydrometallurgists). Smoking was independently associated with having an occupational disease claim in all groups (p < 0.001), as was the number of non-work-related diseases in six groups of seven. Despite consistent improvement in the exposure control measures in nickel production, occupational morbidity persists. More effort is needed to reduce exposure in final product cleaners. © 2022, The Author(s).