- Background Inherited platelet deficiency and/or dysfunction may be more common in the general population than has previously been appreciated. In 2013 the Israeli Inherited Platelet Disorder (IPD) Registry was established. Methods Clinical and laboratory data were collected to pre-specified registration forms. The study protocol was approved by the local hospital ethics committees. Results To date we have included in the registry 89 patients (male 52%) from 79 families. Most patients (74%) have a not-yet specified inherited thrombocytopenia (n = 39) or non-specific platelet function disorder (n = 27). Full clinical data were available for 81 (91%) patients. The median (range) age at presentation and time of follow-up were 1.8 years (1 day–17.8 years) and 4.7 (0–26) years, respectively. The Pediatric Bleeding Questionnaire was available for 78 patients; abnormal bleeding score (≥ 2) was recorded in 47 (52.8%, 95% CI 42%–63.5%) patients and was less frequent in patients followed for isolated thrombocytopenia. Abnormal score was associated with a longer time of follow-up, OR 1.19 (95% CI 1.04–1.36). Conclusion Long term follow-up of patients with IPDs is important as bleeding risks may increase with time. We expect that clinical and laboratory information of patients/families with IPDs gathered in a systemic format will allow for better diagnosis and treatment of these patients.