Objective Auto-inflammatory diseases (AIDs) result from mutations in genes of the innate immune system leading to periodic multisystemic inflammation. We aimed to describe the clinical, biological, molecular features when available and outcomes of Moroccan patients with AIDs. Methods Patient data were retrospectively collected and analyzed over a 13-year period. Results Among 30 Patients, 60% had familial Mediterranean fever (FMF), 16% had mevalonate kinase deficiency (MKD) and 24% other AIDs. The mean age at first consultation was 6.9 years and the mean diagnostic delay was 3 years. Consanguinity was reported in 16 cases. Immunoglobulin A vasculitis was associated in 33% of FMF patients in whom the main clinical features were fever 88.8%, abdominal pain 100%, arthralgias 88.8% and arthritis 50%, and the most frequent mutation M694V 66%. All FMF patients were treated with colchicine. Most MKD patients were confirmed by elevated urinary mevalonic acid levels and 4/5 MKD received targeted therapy. Chronic recurrent osteomyelitis (CRMO) patients were confirmed by radiological and histological analysis. Two cases of Marshall syndrome were diagnosed according to validated criteria. The Familial pustular psoriasis (FPP) case was diagnosed based on histological analysis and the Muckle Wells Syndrome patient by clinical features. The outcome was favorable in 76%, partial in 13% and 3 deaths were reported. Conclusion FMF and MKD are the most reported diseases. AIDs are probably underestimated as they are unknown to clinicians. The aim of this work is to raise awareness among pediatricians about AIDs and create a network for best practices. Lay summary What does this mean for patients ? Autoinflammatory diseases (AIDs) are rare disorders caused by mutations in the genes of the immune system. We retrospectively analyzed data from Moroccan patients with AIDs in a pediatric rheumatology unit. We found a high rate of the M694V genetic mutation in these patients due to marriage between blood relatives, which is common in Moroccan culture. Patients from such marriages are likely to have inherited genetic diseases like Familial Mediterranean Fever (FMF), followed by mevalonate kinase deficiency (MKD) in our cohort. This study represents the first experience of AIDs in Morocco. Diagnosis of AIDs is based primarily on clinical and biological signs as genetic tests are not widely available. This study aims to raise awareness of AIDs among pediatricians and help promote better disease management. This will enable an earlier diagnosis and help to improve the prognosis of the patient.